I'm fairly new to the forums and don't post a lot, but I've been playing video game music on piano for a few years now! I love seeing all the new sheets being added to the site and I like to try to learn as many as possible!
Love me some Mad Jack. So many octaves though, that's terrifying.
Damn, dude. Hell of a lot better than I can play. Keep em coming.
Did you say all of these arrangements were from our site, btw? It's always awesome to see people actually making use of our sheets; we can keep track of download numbers and everything, but to see them actually played really reaffirms the purpose of what we do, at least for me.
Creativity is of no interest, that's why. Just gonna bump this so I do not forget to check when getting home.
Wow. Looks like someone might finally be able to play Bespinben's arrangements.
N's Farewell, In the Hands of Fate, File Select, and Lavender Town are relatively easy to play. Maybe even Trainer Battle from RBY if you simplify it a bit(I can't play it note for note).
I don't have any vids of me playing piano, I have no idea what you're talking about.
You're a great pianist!! I kinda wish the quality was better to reaaaally appreciate your technique. Nonetheless, you sound great. You capture the style very well.
I'm actually trying to learn his arrangement of the Pokemon Ruby/Sapphire title theme right now. It's pretty tough, no question about it. But I'm getting there, I just have trouble with the last part when the key changes to the key of Gb I believe. I prefer to learn "harder" songs, because I like them to sound as accurate as possible to the real thing.
And glad all the people here enjoy my playing!
Another one I'm currently working on is a song from DK Country Returns that I happened to find sheet music for just last week. It's fairly easy, I almost have it memorized!
The youtube one was from another site that I don't think exists anymore, but all the other songs I played were from this site. I sometimes add extra stuff to some of the songs if I feel they don't sound right, but all of the facebook linked ones are from here. A few I had to look up in the topics on the forum, and a few I found on the main site.
I just added a new song! The Main Theme from Jetpack Joyride!!
I'm always stalking the forums looking for new stuff, I just don't sign in ever lol.
I added a bunch of new songs since I haven't signed on here for a month or so!
The newest ones I've added are Wario Land Shake It: Just Plains, and 2 Banjo-Kazooie songs: Freezeezy Peak and Mad Monster Mansion!


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Research ArticleGeneticsOphthalmology Open Access | 10.1172/jci.insight.162687
A pathogenic in-frame deletion-insertion variant in BEST1 phenocopies Stargardt disease
Masha Kolesnikova,1,2 Jin Kyun Oh,3 Jiali Wang,3 Winston Lee,3,4 Jana Zernant,3 Pei-Yin Su,3 Angela H. Kim,1,2 Laura A. Jenny,1 Tingting Yang,3 Rando Allikmets,3,5 and Stephen H. Tsang1,3,5,6
1Jonas Children's Vision Care and Bernard and Shirlee Brown Glaucoma Laboratory, Columbia University, New York, New York, USA.
2SUNY Downstate Health Sciences University, New York, New York, USA.
3Department of Ophthalmology,
4Department of Genetics and Development, and
5Department of Pathology and Cell Biology, Columbia University, New York, New York, USA.
6Institute of Human Nutrition, Columbia Stem Cell Initiative, New York, New York, USA.
Address correspondence to: Stephen H. Tsang, Edward S. Harkness Eye Institute, New York-Presbyterian Hospital/Columbia University Irving Medical Center, 635 West 165th Street, Box 112, New York, New York 10032, USA. Phone: 212.342.1186; Email: sht2@columbia.edu.
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Published October 20, 2022 - More info
Published in Volume 7, Issue 23 on December 8, 2022
JCI Insight. 2022;7(23):e162687. https://doi.org/10.1172/jci.insight.162687.
© 2022 Kolesnikova et al. This work is licensed under the Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.
Published October 20, 2022 - Version history
Received: June 14, 2022; Accepted: October 18, 2022
Here, we describe affected members of a 2-generation family with a Stargardt disease–like phenotype caused by a 2–base pair deletion insertion, c.1014_1015delGAinsCT;p.(Trp338_Asn339delinsCysTyr), in BEST1. The variant was identified by whole-exome sequencing, and its pathogenicity was verified through chloride channel recording using WT and transfected mutant HEK293 cells. Clinical examination of both patients revealed similar phenotypes at 2 different disease stages that were attributable to differences in their age at presentation. Hyperautofluorescent flecks along the arcades were observed in the proband, while the affected mother exhibited more advanced retinal pigment epithelium (RPE) loss in the central macula. Full-field electroretinogram testing was unremarkable in the daughter; however, moderate attenuation of generalized cone function was detected in the mother. Results from electrooculogram testing in the daughter were consistent with widespread dysfunction of the RPE characteristic of Best disease. Whole-cell patch-clamp recordings revealed a statistically significant decrease in chloride conductance of the mutant compared with WT cells. This report on a mother and daughter with a BEST1 genotype that phenocopies Stargardt disease broadens the clinical spectrum of BEST1-associated retinopathy.
BEST1, located on chromosome 11q13, encodes the bestrophin-1 protein, a transmembrane calcium-sensitive chloride channel located in the retinal pigment epithelium (RPE) (1). The alteration of bestrophin results in fluid and lipofuscin accumulation beneath the retina, leading to serous neurosensory detachment and secondary degeneration of photoreceptors (2). Nearly 500 variants have been identified in the BEST1 gene; these variants lead to a wide variety of phenotypic associations collectively known as the bestrophinopathies, which include adult-onset vitelliform macular dystrophy, autosomal dominant vitreoretinochoroidopathy, both autosomal recessive and autosomal dominant bestrophinopathy, bull's eye maculopathy, retinitis pigmentosa, and microcornea, rod-cone dystrophy, cataract, posterior staphyloma syndrome (1, 3).
Best vitelliform macular dystrophy (BVMD), also known as Best disease, is an autosomal dominant inherited retinal dystrophy that occurs in roughly 1 in 15,000–20,000 individuals (4). There are multiple reports of incomplete penetrance in BVMD (5–7). Typically, BVMD presents early in life with fundus findings of vitelliform, or egg yolk-like lesions, that later progress to RPE atrophy; however, the age of onset can be variable, with cases of disease onset as late as 75 years old (8). These patients will often have unperturbed visual acuity early on (2), and up to 5% of patients with a genetic diagnosis of BVMD never develop symptoms or typical fundus findings (8). At more advanced stages, visual deterioration occurs along with the onset of metamorphopsia (2). Nonetheless, visual acuity is often preserved in at least one eye throughout the natural history of disease progression (8). The most sensitive diagnostic test for BVMD is the electrooculogram (EOG). It will show diminished light rise before the onset of ocular symptoms, resulting in a decreased Arden ratio, which will even be abnormal in patients without evident fundus findings (2, 3, 9). A full-field electroretinogram (ffERG), which is frequently used to diagnose other retinal dystrophies, is normal in patients with BVMD.
Given the significant genetic variability responsible for the bestrophinopathies, it has been previously suggested that it is difficult to assess genotype-phenotype correlations; however, others have remained more optimistic (2). To date, a wide variety of clinical presentations have been associated with pathogenic variation in BEST1, and these continue to expand. This report describes what we believe to be a novel phenotype of the BEST1 variant, c.1014_1015delGAinsCT;p.(Trp338_Asn339delinsCysTyr), as seen in a mother and daughter with BVMD phenocopying Stargardt disease.
Case description. A 37-year-old woman (P1) presented to the medical retina clinic at the Columbia University Irving Medical Center for evaluation, with a referring diagnosis of Stargardt disease. The patient was asymptomatic, stating that she was referred based on incidental findings seen during a routine dilated fundus examination. The patient reported a family history of age-related macular degeneration (AMD) in her mother, maternal uncle, and maternal grandfather. At initial presentation, her visual acuity was best corrected to Snellen 20/20 in both eyes. Anterior segment examination was unremarkable. Dilated fundus examination revealed a pattern of yellow pisciform flecks across the macula, sparing the juxta-papillary region in both eyes. The foveal region exhibited a hyperpigmented appearance but was otherwise healthy (Figure 1A). Short-wavelength autofluorescence (SW-AF) revealed hyperautofluorescent flecks along the arcades, extending centrally toward the macula with peripapillary sparing (Figure 1B). Rare central hypoautofluorescent lesions were seen surrounding the fovea bilaterally. Spectral domain–optical coherence tomography (SD-OCT) revealed parafoveal outer retinal atrophy and abrupt disruption of the photoreceptor-attributable ellipsoid zone band in the right eye and retinal thinning of the outer nuclear layers temporal to the fovea, with attenuation of the ellipsoid zone in the left eye (Figure 1, C and D). Axial lengths were measured to be 24.09 mm in the right eye and 24.22 mm in the left eye. ffERG testing showed no generalized rod and cone dysfunction in both eyes (Figure 2A). EOG showed diminished light rise bilaterally, with an Arden ratio of 1.51 in the right eye and 1.47 in the left (Figure 2B).
Yellow pisciform flecks in a mother and daughter with an in-frame deletion-insertion variant in BEST1, as seen using fundus photography and spectral-domain optical coherence imaging. (A) Color fundus image of the left eye of a 37-year-old woman, showing central hyperpigmentation in the fovea and yellow pisciform flecks along the arcades with sparing of the central fovea. (B) Short-wave fundus autofluorescence (SW-FAF) imaging of the left eye, demonstrating a similar pattern of hyperautofluorescent flecks along the arcades, extending centrally toward the macula as well as several hypoautofluorescent lesions surrounding the fovea bilaterally. (C and D) Spectral domain optical coherence tomography (SD-OCT) imaging, showing parafoveal retinal thinning of the outer nuclear layers and attenuation of the ellipsoid zone. (E) Color fundus image of the left eye of the 69-year-old mother of the person in A, showing substantial bilateral retinal pigment epithelium loss in the central macula up to the arcades and relative peripapillary sparing. (F) SW-FAF imaging of the left eye, revealing extensive macular atrophy with a surrounding pattern of fleck extending into the periphery. (G and H) SD-OCT imaging, showing extensive loss of retinal architecture and extensive retinal atrophy with hypertransmission into the choroid.
Electrooculogram and full-field electroretinogram testing results from a mother and daughter with an in-frame deletion-insertion variant in BEST1. (A) Full-field electroretinogram (ffERG) of the daughter (P1) revealed fully preserved scotopic rod-specific, maximum, and photopic cone single flash and 30 Hz flicker responses. ffERG of the mother (P2) revealed that scotopic rod-specific response ERG b-wave amplitudes were 133 microvolts in the right eye and 155 microvolts in the left eye. Maximal ERG a-wave and b-wave amplitudes were 94 and 84 microvolts in the right eye and 120 and 117 microvolts in the left eye. Transient photopic ERG b-wave amplitudes and implicit times were 87 microvolts and 32 milliseconds in the right eye, 106 microvolts and 33 milliseconds in the left eye. Photopic 30 Hz flicker ERG had implicit times and amplitudes of 77 microvolts and 30 milliseconds in the right eye, 99 microvolts and 31 milliseconds in left eye. A normal 43-year-old control is shown for comparison. (B) Electrooculogram (EOG) of the daughter, showing diminished light rise bilaterally with Arden ratios of 1.51 and 1.47 in the right and left eyes, respectively. (C) EOG of the mother was affected by poor fixation but showed diminished light rises, with Arden ratios of 1.28 in the right eye and 1.34 in the left eye.
The affected mother (P2) was a 69-year-old woman previously diagnosed with age-related AMD at 45 years of age. Her visual acuity was best corrected to count fingers at 3 feet in the right eye and 20/250 in the left eye. She reported a family history of AMD in her father and brother who experienced an onset of visual symptoms at ages 60 and 45 years, respectively. Anterior segment examination was remarkable for trace nuclear sclerosis (NS +1) in both eyes. Dilated fundus examination revealed a substantial area of bilateral RPE loss in the central macula extending to the arcades (Figure 1E). SW-AF revealed extensive macular atrophy, with a surrounding pattern of flecks extending in the periphery and relative peripapillary sparing (Figure 1F). SD-OCT showed profound thinning, extensive loss of retinal architecture, and complete outer retinal atrophy with hypertransmission in the choroid (Figure 1, G and H). ffERG revealed relatively unaffected dark-adapted rod-specific responses, with a low B-to-A ratio on maximum responses bilaterally (Figure 2A). Single flash cone and 30 Hz flicker responses showed preserved amplitudes without implicit time delay in either eye. EOG, although affected by poor fixation, revealed diminished light rise bilaterally, with reduced Arden ratios of 1.28 and 1.34 in the right and left eyes, respectively (Figure 2C).
Exome sequencing analysis. Whole-exome sequencing was performed in the proband, and variant filtering was restricted to nonsynonymous exonic and canonical splice site variants in genes previously associated with retinal disease (RetNet; https://sph.uth.edu/retnet/, accessed May 2022). Fifteen heterozygous variants were identified with a minor allele frequency (MAF) equal to or less than 0.005 (Table 1). Rare and predicted pathogenic variants were identified in several genes, including a canonical splice site variant, c.97-2A > G (SpliceAI Δscore =1.00), in NEK2, missense variants in PLK4, EYS, RPGRIP1L, DMD, and BEST1; however, all except the latter, are associated with autosomal or X-linked recessive retinal diseases. Interestingly, no pathogenic variant was identified in the ABCA4 gene, the causal gene for Stargardt disease (Supplemental Table 1; supplemental material available online with this article; https://doi.org/10.1172/jci.insight.162687DS1).
Genetic and functional annotation summary of rare variants (MAF ≤ 0.005) in genes identified by whole-exome sequencing in the proband
The BEST1 (NM_004183.4) variant is a 2 bp in-frame deletion-insertion (c.1014_1015delGAinsCT;p.(Trp338_Asn339delinsCysTyr)) of 2 highly conserved nucleotides (ref. 10; phyloP100way > 9) that code for 2 residues in the intracellular domain of the protein. The variant is absent from the general population (i.e., ultrarare) according to the gnomAD database and is predicted deleterious by deletion-insertion–specific pathogenicity algorithms: MutPred-InDel (ref. 11; g = 0.63) and PROVEAN (ref. 12; score = –18.81). When analyzed as individual variants, the p.(Trp338Cys) and p.(Asn339Tyr) substitutions are also universally predicted to be highly pathogenic across SNV-specific algorithms. Direct sequencing of exon 9 of BEST1 in the affected mother confirmed maternal segregation of this variant. In the proband, no putatively pathogenic ABCA4 variants were identified at a MAF filter of less than or equal to 0.005. Several intronic, synonymous, and missense ABCA4 variants present at an MAF threshold of less than or equal to 0.1 (Supplemental Table 1); although, as expected, none are predicted to have any contributing effect in this case.
Functional testing of BESTTrp338_Asn339delinsCysTyr. Electrophysiological analyses of whole-cell patch-clamp recordings revealed decreased density-voltage relationships between HEK293 cells expressing WT and mutant human BEST1 (Figure 3). Two-tailed unpaired Student's t tests between WT and mutant HEK293 cells suggested significant differences in chloride conduction (P < 0.05).
Diminished chloride conductance in cells expressing Trp338_Asn339delinsCysTyr. Population steady-state current density-voltage relationships at 1 μM Ca2+ in HEK293 cells expressing WT (black) and mutant (white) human BEST1. n = 5–6 for each point. *P < 0.05 compared with currents from WT, using 2-tailed unpaired Student's t test.
The variability in phenotype has been a topic of interest, with many reports working to assess a genotype-phenotype correlation and documenting unusual presentations, such as those with widespread flecks in the midperiphery (8) and bone spiculation in the far periphery reminiscent of retinitis pigmentosa (13). This case report further expands the known phenotypes of BEST1 by describing a mother and daughter who were found to have genetic diagnosis of BEST1, c.1014_1015delGAinsCT;p.(Trp338_Asn339delinsCysTyr) and presented with fundus findings of extensive pisciform fleck and central macular atrophy phenocopying Stargardt disease. This variant causes the substitution of 2 highly conserved cytoplasmic bestrophin residues (14) and was shown to be pathogenic in our functional modeling. BEST1 functions selectively as a calcium-gated chloride channel within the RPE, and targeted testing of chloride conductance revealed decreased conductance in transfected HEK293 cells in comparison with WT cells. This was more suggestive of a loss-of-function mutation as opposed to gain of an alternative function.
This rare variant has been described only once previously in a compound heterozygous patient with a diagnosis of autosomal recessive bestrophinopathy (ARB) (15). A number of BEST1 variants, such as the c.884_886delTCA;p.(Ile295del) (15) and c.422G > A;p.(Arg141His) (16, 17) variants, have been associated with both BVMD and ARB. We suggest that the c.1014_1015delGAinsCT;p.(Trp338_Asn339delinsCysTyr) variant may be included in the list of variants associated with both BVMD and ARB.
Numerous inherited retinal dystrophies that phenotypically mimic Stargardt disease have been previously reported, including those associated with variants in ELOVL4, PROM1, RDS/PRPH2, and CLN3 (18). Variants in BEST1 have been frequently suggested to also be responsible for a Stargardt disease–like degeneration, owing to a shared pathophysiology (19–21). However, there have been only two prior reports of BEST1 presenting with fleck-like lesions: a 54-year-old man with fleck nasal to the disc and along the temporal arcades (9) and a 74-year-old-man presenting with midperipheral fleck late in life (8).
P1 presented with fundus findings of yellow pisciform flecks extending anterior to the arcades and nasal to the optic disc, reminiscent of a Stargardt disease phenotype (Figure 4A) (18). The SW-AF images of P1 showed a similar pattern to the SW-AF pattern seen in Fishman group II Stargardt disease, demonstrating hyperautofluorescent flecks along the arcades, extending centrally toward the macula (Figure 4B) (22). Although the clinical presentation was consistent with Stargardt disease, both CLIA and extensive research genetic testing revealed no variants in ABCA4 or other known phenocopies, and EOG results revealed an abnormal light rise consistent with BVMD. ffERG revealed normal scotopic rod-specific and photopic cone-specific responses, which would be expected in both Stargardt disease and BVMD (23). In contrast, P2 presented with a fundus phenocopy of a more advanced Stargardt disease phenotype, likely attributable to the difference in age between the patients. The fundus findings revealed severe macular atrophy, extending to the arcades, and SW-AF demonstrated similar patterns of macular atrophy invading the arcades, comparable to Fishman group III, between P2 and a patient with a confirmed diagnosis of Stargardt disease (Figure 4, C and D) (22). This patient was also found to have the same heterozygous variant in BEST1. EOG, although affected by the patient's poor fixation, showed diminished light rise bilaterally, and ffERG revealed preserved scotopic rod-specific responses with a low B-to-A ratio, suggesting inner retinal dysfunction consistent with that in published literature (24–26) and diminished photopic-cone single flash and 30 Hz flicker responses. While ffERG findings are often normal in early BVMD and Stargardt disease, diminished scotopic and photopic responses can be expected in later-stage Stargardt disease (23). Only patients diagnosed with multifocal BVMD, the autosomal recessive form of the disease (27), will show abnormal ffERG results (28, 29).
BEST1Trp338_Asn339delinsCysTyr phenocopies Stargardt disease on fundus images and fundus autofluorescence imaging. (A) A color fundus image of a 37-year-old woman with BVMD (left) and a color fundus image of a 45-year-old woman with a confirmed diagnosis of Stargardt disease (right), revealing similar patterns of yellow pisciform flecks, extending anterior to the arcades and nasal to the optic disc. (B) Short-wavelength imaging similarly shows similar patterns of hyperautofluorescent flecks along the arcades, extending centrally toward the macula between a 69-year-old woman with BVMD (left) and a patient with Stargardt disease (right). (C) Color fundus image and (D) short-wavelength autofluorescence imaging of a 69-year-old woman with BVMD (left) and images of an 80-year-old man with severe Stargardt disease (right), demonstrating severe macular atrophy approaching the arcades.
A possible explanation for the phenotypic similarity between the BVMD diagnosed in these patients and Stargardt disease lies in the histopathologic findings. Petrukhin et al. (21) have shown that both BVMD and Stargardt disease are associated with lipofuscin accumulation, and others have similarly suggested that BVMD may share a pathophysiology with Stargardt disease (30–32). Lipofuscin is a complex aggregate of cellular components that is found in various cells throughout the body; in the RPE specifically, lipofuscin accumulation results from phagocytosis of the photoreceptor outer segments (33). Extensive study of the lipofuscin accumulation in BVMD has demonstrated increased levels of A2E, the same component of lipofuscin that has been found in high levels in Stargardt disease (30, 31). Although the exact mechanism by which A2E accumulation occurs in BVMD is unknown, it has been suggested that impairment of the bestrophin calcium-sensitive chloride channels leads to loss of both phagolysosome acidification and regulation of vesicle fusion, either of which may cause the subsequent accumulation of lipofuscin (30).
Variants in BEST1 are a well-described cause of inherited retinal dystrophy, with a continually expanding variety of phenotypes. In this report, we described the case of a mother and daughter presenting with BVMD phenocopying Stargardt disease caused by a heterozygous pathogenic variant in BEST1. Given the possible novelty of the variant and the phenotype, future studies of the functional impact of this variant as well as additional genotype-phenotype correlation in larger cohorts may help clarify the similarity in disease pathophysiology between BVMD and Stargardt disease.
Clinical evaluation. Two patients were evaluated at the Edward S. Harkness Eye Institute at the Columbia University Medical Center. Patients underwent complete ophthalmic examination, beginning with dilation using topical tropicamide (1%) and phenylephrine hydrochloride (2.5%). Imaging studies were conducted using SD-OCT (Spectralis HRA2, Heidelberg Engineering), SW-AF (Spectralis HRA2, Heidelberg Engineering), and wide-angle color fundus image using an Optos 200Tx unit (Optos). ffERG and EOG were conducted using the Diagnosys Espion Electrophysiology System. ffERG was performed according to ISCEV standards (34).
Exome sequencing and analysis. Genomic DNA was extracted from peripheral blood lymphocytes extracted from whole blood from the proband. Whole-exome sequencing was performed by Psomagen using the SureSelect Human All Exon V8 (Agilent). Sequencing reads were aligned to the hg19 reference genome using Burrows-Wheeler Alignment tool and processed with GATK according to the best practices recommendations (35). After variant calling, we narrowed our analyses to variants in genes previously associated with retinal disease (RetNet; https://sph.uth.edu/retnet/) at a MAF of equal to or less than 0.005 according to the gnomAD database (https://gnomad.broadinstitute.org/; accessed May 2022). We then performed functional annotation on the called variants with ANNOVAR (36) using pathogenicity scores from the dbnsfp 4.2a data set (37). Pathogenic effects on splicing were assessed using SpliceAI (38). Deletion-insertion-specific pathogenicity predictions were analyzed by SIFT_indels2 (39) and PROVEAN (40).
Functional testing of BESTTrp338_Asn339delinsCysTyr. Electrophysiological analyses were conducted 48–72 hours after transfection. Whole-cell patch-clamp recording was performed with an EPC10 patch-clamp amplifier (HEKA Electronics) controlled by Patchmaster (HEKA). Micropipettes were pulled and fashioned from filamented 1.5 mm thin-walled glass (WPI Instruments) and filled with internal solution containing 130 mM CsCl, 1 mM MgCl2, 10 mM EGTA, 2 mM MgATP (added fresh), and 10 mM HEPES (pH 7.4, adjusted by CsOH). The desired Ca2+ concentrations were obtained by adding CaCl2 (Ca/Mg/ATP/EGTA Calculator v1 was used to calculate CaCl2, https://somapp.ucdmc.ucdavis.edu/pharmacology/bers/maxchelator/CaMgATPEGTA-TS.htm). Series resistance was typically 1.5–2.5 MΩ, with no electronic series resistance compensation. The recipe of external solution was 140 mM NaCl, 5 mM KCl, 2 mM CaCl2, 1 mM MgCl2, 10 mM HEPES (pH 7.4, adjusted by NaOH), and 15 mM glucose. Solution osmolarity was 290~310 mOsm/L with glucose. Traces were acquired at a repetition interval of 4 seconds. Currents were sampled at 25 kHz and filtered at 5 or 10 kHz. I-V curves were generated from a group of step potentials (–100 to +100 mV from a holding potential of 0 mV). Experiments were conducted at room temperature (23°C ± 2°C). Whole-cell patch-clamp data were processed off-line in Patchmaster.
Statistics. Statistical analyses were performed using built-in functions in Origin and 2-tailed unpaired Student's t test. P values of less than 0.05 were considered significant.
Study approval. All procedures reviewed were in accordance with the tenets of the Declaration of Helsinki. Studies were reviewed and approved by the Columbia University Institutional Review Board (protocol AAAB6560). Written informed consent was obtained from patients, as regulated by the Columbia University Institutional Review Board. Written informed consent was obtained for all clinical images published with this manuscript.
SHT, TY, and RA designed the research studies. JW and TY conducted the experiments. MK, JW, TY, WL, and RA acquired the data. MK, JKO, JW, TY, WL, and RA analyzed the data. JZ performed genetic sequencing. PYS recruited patients. JW and TY provided reagents. MK and JKO wrote and prepared the original draft. WL, LAJ, and AHK reviewed and edited the manuscript.
View Supplemental table 2
We thank the Jonas Children's Vision Care for sharing ideas and for critically reading the manuscript. This study was supported, in part, by NIH grants 5P30CA013696, U01 EY030580, U54OD020351, R24EY028758, R24EY027285, 5P30EY019007, R01EY018213, R01EY024698, R01EY026682, R01EY028203, R01EY029315, R21AG050437, GM127652, EY028758, and 1U01EY034590; the Irma T. Hirschl/Monique Weill-Caulier Award (HRSCHL CU20-4313); the Schaefer Research Award; the Schneeweiss Stem Cell Fund; New York State (SDHDOH01-C32590GG-3450000); the Foundation Fighting Blindness New York Regional Research Center Grant (TA-NMT-0116-0692-COLU); Foundation Fighting Blindness (award PPA-1218-0751-COLU); Nancy and Kobi Karp; the Crowley Family Fund; the Rosenbaum Family Foundation; the Alcon Research Institute; the Gebroe Family Foundation; the Research to Prevent Blindness (RPB) Physician-Scientist Award; and the unrestricted funds from the RPB to the Department of Ophthalmology, Columbia University.
Conflict of interest: SHT receives financial support from Abeona Therapeutics Inc. and Emendo. He is also the founder of Rejuvitas and is on the scientific and clinical advisory boards for Nanoscope Therapeutics.
Copyright: © 2022, Kolesnikova et al. This is an open access article published under the terms of the Creative Commons Attribution 4.0 International License.
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Haji S, et al. Stargardt-Fundus flavimaculatus: recent advancements and treatment. Semin Ophthalmol. 2013;28(5–6):372–376.
Petrukhin K, et al. Identification of the gene responsible for Best macular dystrophy. Nat Genet. 1998;19(3):241–247.
Fishman GA, et al. Variation of clinical expression in patients with Stargardt dystrophy and sequence variations in the ABCR gene. Arch Ophthalmol. 1999;117(4):504–510.
Huang D, et al. Stargardt disease and progress in therapeutic strategies. Ophthalmic Genet. 2022;43(1):1–26.
Power WJ, et al. The pattern ERG in Best's disease. Doc Ophthalmol. 1990;76(3):279–284.
Scholl HP, et al. Mapping of retinal function in Best macular dystrophy using multifocal electroretinography. Vision Res. 2002;42(8):1053–1061.
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Preising MN, et al. [Autosomal recessive bestrophinopathy (ARB): a clinical and molecular description of two patients at childhood] [German]. Klin Monbl Augenheilkd. 2012;229(10):1009–1017.
Jarc-Vidmar M, et al. Fundus autofluorescence imaging in Best's vitelliform dystrophy. Klin Monbl Augenheilkd. 2003;220(12):861–867.
Alba Linero C, et al. Multifocal Best's disease: the importance of genetic tests. Arch Soc Esp Oftalmol (Engl Ed). 2018;93(3):136–138.
Bakall B, et al. Enhanced accumulation of A2E in individuals homozygous or heterozygous for mutations in BEST1 (VMD2). Exp Eye Res. 2007;85(1):34–43.
Zhang Y, et al. Suppression of Ca2+ signaling in a mouse model of Best disease. Hum Mol Genet. 2010;19(6):1108–1118.
Boon CJ, et al. Clinical and molecular genetic analysis of best vitelliform macular dystrophy. Retina. 2009;29(6):835–847.
Sparrow JR, Boulton M. RPE lipofuscin and its role in retinal pathobiology. Exp Eye Res. 2005;80(5):595–606.
McCulloch DL, et al. ISCEV standard for full-field clinical electroretinography (2015 update). Doc Ophthalmol. 2015;130(1):1–12.
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SITA acquires self-service bag-drop manufacturer Type22
By Helen Norman	 December 2, 2015 No Comments
Air transport IT provider SITA has today (December 2) announced the acquisition of Dutch-based self-service bag-drop developer Type22.
The acquisition will strengthen SITA's end-to-end technology solutions by adding Type22's self-service bag-drop portfolio, including its Scan&Fly and Drop&Fly solutions.
Francesco Violante, CEO of SITA, said, "SITA has been providing its Self BagDrop solution since 2010, with several successful deployments worldwide. According to SITA's 2015 Airport IT Trends Survey, 62% of passengers will use self-service bag-drop by 2017. Recognizing the tremendous potential in the market made acquiring Type22 a logical next step that will make us leaders in self-service bag-drop integrated with common-use systems. The addition of Type22's product portfolio and expertise will provide SITA's customers with automated self-service bag-drop solutions that are perfect for both new airport terminals, as well as retrofitted into existing desk environments."
Roel Reijnen, managing director of Type22, said, "At Type22 we have been entirely focused on self-service bag-drop solutions since 2011 and our team has the technical experience as well as a keen understanding of the needs of the customer. With an established strong customer base in Europe and Asia, we look forward to continued growth in these and other markets, adding to the SITA success story."
SITA will continue to develop Type22's products and will provide support for all of the company's existing customers. Members of Type22's staff will remain located in Delft, in the Netherlands.
Helen Norman, editor-in-chief
Helen has worked for UKi Media & Events for nearly a decade. She joined the company as assistant editor on Passenger Terminal World and since progressed to become editor of five publications, covering everything from aviation, logistics and e-commerce to meteorology. She has a love for travel and property and has redeveloped three houses in three years. When she's not editing magazines, she's running around after her two boys and their partner in crime, Pete the pug.
Swissport expands Aspire lounge business to Asia
Geneva Airport to inaugurate sustainable East Wing in spring 2022


Monday morning rolls around and you are crushing life. You are meal prepped for the week, start your work day without spilling coffee on yourself, and make it to your afternoon workout. Throughout the week, you stay on track with your nutrition and are feeling really good. You started going to at a new gym and are starting to see familiar faces. You are on your GAME.
And then, you go out of town for a long Memorial Day Weekend. You overindulge on food and drinks, aren't as active as you have been, and didn't have a chance to go to the grocery store and meal prep for the week. It's all of a sudden the end of the week and you haven't had any movement since before your vacation. Do you have to start over completely? How can you STAY active when life gets in the way? I'm here to help!
1. Find the right motivation/inspiration.
I've written about this before here but I do believe that you have to want to do something for all the right reasons. Do you want to live longer? Do you want to be able to hang with your kids all day? Do you want to walk up the stairs and feel good? There can be many different motivating factors, but they have to come from a place of positivity and eagerness to make it work. No matter what it is, post it on your mirror and think about it every single day.
Do you know that you are going to happy hour after work with your co-workers or that you are going to be out of town for a couple days? PLAN for it! I always take a set of clothes, Secret Active Deodorant, and food if I am going to be out all day. Being prepared will 100% make life and staying active easier. Going out of town? Get your groceries delivered for the day you get back!
Working out and staying active is so much more fun with friends. It holds you accountable, makes it more fun, and you are more likely to sweat! I love to use Secret Active when I'm working out because it keeps me feeling and smelling fresh even though I am the sweatiest in the group!
4. Choose activities that you LOVE.
This is important. You can't be going around forcing yourself to run when you aren't and will never be a runner. Sign up for something that you are going to be excited about and look forward to. That way it won't feel like a chore and it will actually just be fun!
5. Move your body throughout the day.
Every single day, look for different ways you can move. Whether that is taking the five flights of stairs or parking in the last spot, always look for ways that you can add a little bit of extra movement. On a conference call and not talking much? Do some squats in your chair while you are waiting. Waiting for a flight at the airport? Do lunges until you hear another name over the loud speaker then switch to squats. There are so many "silly" ways you can move your body that can actually help you be more active throughout the day!
One of the moments I feel the most "active" is when I am sweating, and I wouldn't be able to feel as good as I do while sweating if it wasn't for Secret Active. It was specifically formulated for high activity, so the more I sweat, the more odor protection I get. Which is honestly so great for sweaty me. If you're like me, head over to Target to try Secret Active out yourself!
Tell me : What is your number one tip to stay active when it's really hard to do so?
This post is brought to you by Secret Active. A product I use daily and really love! This post contains some affiliate links and I may be compensated for this post, but all opinions are my own. This compensation helps with expenses to keep this blog up and running!


Total announced that its subsidiary Total E&P Nigeria Ltd. (TEPNG) has made a new oil discovery in the southeastern corner of Oil Mining Lease (OML) 102 in Nigeria. The discovery is located 65 kilometers off the southeastern coast of the country, about 15 kilometers southeast of the Ofon field.
The Etisong North-1 well was drilled to a total depth of 2,387 metres in 80 metres of water. One of the three reservoirs encountered tested at 8,500 barrels per day of 40° API oil. This is the second discovery in the lease, following on from the Etisong Main find in 2008, increasing the feasibility of a new development hub on OML 102.
OML 102 is operated by TEPNG with a 40% interest, alongside partner NNPC, which holds the remaining 60%.


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Medical and Health /
Medical Care Economic Risk: Measuring Financial Vulnerability from Spending on Medical Care
Medical Care Economic Risk: Measuring Financial Vulnerability from Spending on Medical Care Sented by Rebecca
The United States has seen major advances in medical care during the past decades, but access to care at an affordable cost is not universal. Many Americans lack health care insurance of any kind, and many others with insurance are nonetheless exposed to financial risk because of high premiums, deductibles, co-pays, limits on insurance payments, and uncovered services. One might expect that the U.S. poverty measure would capture these financial effects and trends in them over time. Yet the current official poverty measure developed in the early 1960s does not take into account significant increases and variations in medical care costs, insurance coverage, out-of-pocket spending, and the financial burden imposed on families and individuals. Although medical costs consume a growing share of family and national income and studies regularly document high rates of medical financial stress and debt, the current poverty measure does not capture the consequences for families' economic security or their income available for other basic needs. In 1995, a panel of the National Research Council (NRC) recommended a new poverty measure, which compares families' disposable income to poverty thresholds based on current spending for food, clothing, shelter, utilities, and a little more. The panel's recommendations stimulated extensive collaborative research involving several government agencies on experimental poverty measures that led to a new research Supplemental Poverty Measure (SPM), which the U.S. Census Bureau first published in November 2011 and will update annually. Analyses of the effects of including and excluding certain factors from the new SPM showed that, were it not for the cost that families incurred for premiums and other medical expenses not covered by health insurance, 10 million fewer people would have been poor according to the SPM. The implementation of the patient Protection and Affordable Care Act (ACA) provides a strong impetus to think rigorously about ways to measure medical care economic burden and risk, which is the basis for Medical Care Economic Risk. As new policies - whether part of the ACA or other policies - are implemented that seek to expand and improve health insurance coverage and to protect against the high costs of medical care relative to income, such measures will be important to assess the effects of policy changes in both the short and long term on the extent of financial burden and risk for the population, which are explained in this report.
Download book (pdf - 10.93 MB)
The United States has seen major advances in medical care during the past decades, but access to care at an affordable cost is not universal. Many Americans lack health care insurance of any kind, and many others with insurance are nonetheless exposed to financial risk because of high premiums, deductibles, co-pays, limits on insurance payments, and uncovered services. One might expect that the U.S. poverty measure would capture these financial effects and trends in them over time. Yet the current official poverty measure developed in the early 1960s does not take into account significant increases and variations in medical care costs, insurance coverage, out-of-pocket spending, and the financial burden imposed on families and individuals. Although medical costs consume a growing share of family and national income and studies regularly document high rates of medical financial stress and debt, the current poverty measure does not capture the consequences for families' economic security or their income available for other basic needs.
In 1995, a panel of the National Research Council (NRC) recommended a new poverty measure, which compares families' disposable income to poverty thresholds based on current spending for food, clothing, shelter, utilities, and a little more. The panel's recommendations stimulated extensive collaborative research involving several government agencies on experimental poverty measures that led to a new research Supplemental Poverty Measure (SPM), which the U.S. Census Bureau first published in November 2011 and will update annually. Analyses of the effects of including and excluding certain factors from the new SPM showed that, were it not for the cost that families incurred for premiums and other medical expenses not covered by health insurance, 10 million fewer people would have been poor according to the SPM.
The implementation of the patient Protection and Affordable Care Act (ACA) provides a strong impetus to think rigorously about ways to measure medical care economic burden and risk, which is the basis for Medical Care Economic Risk. As new policies - whether part of the ACA or other policies - are implemented that seek to expand and improve health insurance coverage and to protect against the high costs of medical care relative to income, such measures will be important to assess the effects of policy changes in both the short and long term on the extent of financial burden and risk for the population, which are explained in this report.
Epilepsy Case Studies: Pearls for Patient Care
Critical Care Sedation
Information Technology in Health Care 2007 - Proceedings of the 3rd International Conference on Information Technology in Health Care: Socio-technical ... Studies in Health Technology and Informatics
Board on Health Care Services
Division of Behavioral and Social Sciences and Education
Committee on National Statistics
Panel on Measuring Medical Care Risk in Conjunction with the New Supplemental Income Poverty Measure
BEA WebLogic Server 8 For Dummies Sented by Shon
Instant Web Scraping with Java Sented by Shon
Java for Everyone: Late Objects Sented by Shon


Zlatan Ibrahimovic has reportedly been forced to take a 95 per cent pay cut to join the L.A. Galaxy, with his wages dropping from €21.8 million per year to €1.2 million.
L.A. Galaxy announced on Friday they had signed the striker after Manchester United had terminated his contract early.
Club president Chris Klein explained why they had invested in the 36-year-old, telling the club's official website: "In addition to our rich history, we continue to invest in building a future that will see us continue to reach new achievements. We aggressively pursued Zlatan and are extremely pleased to add him to our team."
Ibrahimovic was earning £220,000 per week at Manchester United, making him one of the highest-paid players in the Premier League, according to Mick Gadd at the Mirror.
Right decision to allow Ibrahimovic to leave. Age is finally starting to take its toll and United could put his wages to much better use next season.
The striker's arrival should boost the club's shirt sales, as he was in the top 10 of best selling shirts in the Premier League, according to sales recorded by Sports Direct (via Charlotte Duncker at the Manchester Evening News).
Ibrahimovic's arrival at the Galaxy should help bring in the crowds, and although he may well have had to take a pay cut, he's a charismatic character who is unlikely to be short of offers to top up his salary.


People who live with disabilities often face fear, discomfort, and hostilityat a rate that far exceeds that encountered by those who do have no disability. The vast majority of such treatment is rooted in a basiclack of understanding about the challenges that come with having adisability, and the experience of sharing the world with people who do not. People often seek to fill in gaps in their knowledge, and when information is lacking, confusion and even fear may result.
Ask if a person needs assistance before attempting to assist them. All people, whether or not they live with a disability, take pride in what they are able to do. Making any assumption about a person's abilities in any given situation can rob them of this feeling.
Speak directly to a person with a disability, even if he or she has an interpreter. While a person with a hearing impairment may have to look at an interpreter for communication, it is discouraging to everyone to be looked around or over when communicating with someone.
Ask permission before touching and assistive device or service animal. These items and creatures are the tools that the user needs to live their life. They are very important, and very personal.
The more a discussion of etiquette directly relates to lifestyles and personal abilities, the more likely it is that the word "never" is used to illustrate what not to do. While this is often a useful guideline, it can be cumbersome in some situations. In some cases, people who are less experienced in respectful and positive communication may stumble over wording, and significantly impair communication efforts. In other cases, people who are living with a disability may have feelings about language that is not in keeping with the established guidelines for etiquette. Whatever the reason, the commonly recognized best practices of disability-related etiquette may not always be the preferred practices, and it is always most important for the most effective and respectful communication, to first respect the wishes of the individual.
People with mobility impairments are often the most immediately identifiable people who are living with disabilities. As such, the stigma that our culture attaches to these people out of fear and ignorance, often impacts people with mobility impairments most frequently, and most harshly.
Developmental and cognitive disabilities are an extremely broad, but very inter connected category. Both types of disability frequently occur together as a result of a single causal factor, though they just as frequently occur individually. While an experienced and compassionate person may be able to recognize that a person has a developmental disability, there is often no way of knowing whether the person also suffers from a cognitive disability without interacting with that person. Likewise, cognitive disabilities may occur in people who do not appear externally to have any disability whatsoever. It may take observation of behavior and interaction to determine how to communicate most appropriately with someone. Further, a basic recognition that a person's behavior differs from the range that is considered mainstream may not absolutely indicate a cognitive disability, but could be a result of a mental illness (see below) or simply a personality quirk that does not constitute a disability. Lastly, a person with a cognitive disability may not be recognizable in casual interaction. Conditions such as dyslexia, and attention deficit are classified as cognitive disabilities, and can reasonably require both accommodation and sensitivity, but neither will necessarily be immediately apparent. It is in this broad category that the most care must be taken in making assumptions about what aperson is or is not capable of doing on their own or with assistance.
People with Mental Retardation or Cognitive Disabilities Focusing on assumptions about cognitive disability.
Make no assumptions about what someone who is blind can and cannot do. Modern assistive technology has made things that were formerly inaccessible to the blind common place. A primary example is computer usage, which has become a nearly ubiquitous skill for sighted people, and is rapidly becoming standardized as audio screen readers and web standards converge.
Deafness is an extremely common disability, and one which does not present the same mobility issues faced by many other people with disabilities. This combined with modern support for signing (which was once discouraged as a form of communication) has resulted in vibrant deaf communities springing up around the world. Still interacting with a person who is deaf can be challenging, as deafness presents a communication barrier not experienced by most people who live with disabilities.
Communicating with Deaf People: A Primer A comprehensive set of guidelines for hearing people.
People living with mental illness may or may not have a disability. The specific criteria for determining the nature or severity of apsychiatric condition that constitutes a disability constantly shifts, but generally rests on a consideration of the level of impairment of daily activities suffered by the person in question.The biggest barrier faced by people living with mental illness is the lack of understanding which is nearly universal to almost all psychiatric disorders. Stigma and the accompanying ignorance, remains the primary barrier to overcome.
Discribing People With Disabilities A resource on people first language for use when talking about people who have disabilities.
Everyone will at some point make a mistake in conversation. This is one occasion that requires absolutely no special consideration for people who live with disabilities. As when interacting with anyone else,when a mistake is made, simply apologize. People who live with disabilities learn early and unequivocally that others are frequently uncomfortable interacting with them. Most people who have lived with a disability since birth (and many who have not) have experienced bullying and harassment as a result of individual ignorance. A conversational error will not be the harshest experience suffered by any person living with a disability. An apology is an acknowledgement of an error and of a person's intention to be sensitive. Communicating an awareness of etiquette and concern for an individual's feelings may even set one apart from the crowd, and be the first step toward making a new friend.


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This bike bag look so cool. But it was so heavy. When you take your bike to air trip please concern about its weight.
Packing is very easy! Traveling is very easy!! Looks is very cool!!! I'm Japanese. I used this case for my trip from Yokohama to Kuwait. Bus : No extra cost from station to Narita. Fright : No extra cost from Narita to Kuwait by Etihad airways. Compared with normal traveling case, this is Excellent!!!


What have the MAX audience been requesting?
We had a couple of days on our hands over the Queen's Birthday long weekend, and we wanted to make the largest MAX By Request list, so we put the call out.
291. HOODOO GURUS – LIKE WOW WIPEOUT!
228. NAUGHTY BY NATURE – O.P.P.
190. SHANIA TWAIN – MAN! I FEEL LIKE A WOMAN!


"We go outside to rinse ourselves of worldly stress, and to taste again that simple life. In our escape, we leave behind Facebook and Twitter for a brief time, choosing instead face-to-face time beside the campfire, and the tweet of the kingfisher along the riverbank.
We go outside to recharge. To sweat. To ingest a healthy dose of dirt. And learn lessons that cannot be taught in any book or website. We go to test ourselves, and our instincts. To enjoy our friends in a different light. To set our imaginations alight. We go to taste freedom.
Amid those cathedral groves, blue waters and high peaks, we glimpse the insignificance – and the ephemeral beauty – of our own precious life. In the process, we unearth a kinder, gentler incarnation of ourselves. It seems an inescapable truth that we are always a better person for having been outdoors."


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Claire Wong
Claire is a director, actress and also the co-founder of Checkpoint Theatre, a theatre company which is based in Singapore. Since young, Claire has always had a passion for theatre, and was fortunate enough to find herself surrounded by like-minded peers, who encouraged her to pursue her interest. Despite studying law as an undergraduate and eventually starting a career in the legal industry, Claire continued to actively participate in the Singaporean theatre scene and even took a break from the corporate world to further her studies in New York, where she graduated with a Master of Fine Arts in Theatre Arts from Columbia University.
Inspired by the amazing scripts they were getting from local students in a playwriting course taught by her husband, Huzir Sulaiman, Claire and Huzir decided to set up Checkpoint Theatre in 2002 as a sanctuary where Singaporean theatre-makers could grow their craft. Checkpoint Theatre focuses on developing original, thought-provoking works by local playwrights and hopes to nurture the next generation of voices in the local theatre scene. Since its inception, they have staged numerous award-winning plays, and have actively focused on telling stories which are drawn from the Asian context.
Eliza Noordin
Poh Li San


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Yemen: Saudi-led coalition airstrikes target al Houthi-Saleh positions in Sana'a; Hadi government forces clash with al Houthi-Saleh forces in Taiz; al Houthi-Saleh forces claim to shoot down a Saudi helicopter near Najran, Saudi Arabia; al Houthi-Saleh forces launch Zelzal-3 missile towards Najran, Saudi Arabia; Hadi government forces clear AQAP militants from al Wadhea, Abyan
Horn of Africa: U.S. announces $146 million aid package to assist refugees at Dadaab camp in western Kenya; al Shabaab militants ambush AMISOM convoy near Goof Gaduud town, Bay region; Kenya deploys additional forces to secure northern Kenyan-Somali border regions
Saudi-led coalition airstrikes targeted al Houthi-Saleh military camps in central and northeast Sana'a city on August 23. Coalition forces bombed Camp al Siana in central Sana'a city and Camp Kharafi in northeast Sana'a city. The Saudi-led coalition resumed airstrikes on al Houthi-Saleh targets inside Sana'a city on August 9.[1]
Forces loyal to President Abdu Rabbu Mansour Hadi clashed with al Houthi-Saleh forces in Aqaba market, al Salwa district, Taiz governorate on August 23. Hadi government forces began a new offensive on Taiz city following the breakdown of peace talks on August 6.[2]
Al Houthi-Saleh forces claimed to shoot down a Saudi helicopter near Najran city, southern Saudi Arabia on August 23. A local news station aired footage of an object falling from the sky and claimed that it was the helicopter. Saudi Apache helicopters conducted strafing operations along the Yemeni-Saudi border targeting al Houthi-Saleh forces responsible for launching rockets at Najran city on August 22.[3]
Al Houthi-Saleh forces launched a Zelzal-3 ballistic missile towards Najran city on August 23. Pro-al Houthi media reported that the missile launch is a response to Saudi Arabia's continued participation in the bombing campaign in Yemen. [4]
Hadi government forces continued counterterrorism operations in al Wadhea village and other smaller villages throughout Abyan governorate. Al Qaeda in the Arabian Peninsula (AQAP) militants fled from al Wadhea into al Mahfad village. Al Mahfad is AQAP's last known stronghold in Abyan governorate, according to local sources. Security forces discovered a bomb making site in Khanfar Court in Abyan governorate on August 23.[5]
The United States will provide $146 million in aid to Kenya, Somalia, and the UN High Commissioner on Refugees (UNHCR) to assist refugees at Dadaab camp in Kenya. Kenya rescinded its decision to close the camp by the end of 2016 on August 21, likely due to U.S. pressure. U.S. Secretary of State John Kerry announced the planned aid while in Nairobi to discuss security concerns with East African nations.[6]
Al Shabaab militants ambushed an African Union Mission in Somalia (AMISOM) convoy near Goof Gaduud town in Bay region on August 22. The AMISOM forces killed eight al Shabaab militants as they repelled the attack. The militants wounded three AMISOM troops.[7]
Kenya deployed additional security forces along the Kenyan-Somali border in an effort to prevent al Shabaab attacks on critical infrastructure. Al Shabaab militants destroyed telecommunications equipment in Mandera County Kenya in Damasa area on June 25 and Fino town on July 28.[8]
[1] "Raids on camp al Siana center of the capital Sanaa," al Masdar, August 23, 2016, http://almasdaronline.com/article/83999.
[2] "Resistance and troops are attacking Houthi positions south of Taiz control sites," al Masdar, August 23, 2016, http://almasdaronline.com/article/83996.
[3] "Houthis declare dropping a Saudi Apache helicopter in Najran," Yemen Akhbar, August 23, 2016, http://www.yemenakhbar.com/yemen-news/463213.html; and "Najran Army and People's Committees fall hostile Apache helicopter, Yemen TV, August 22, 2016, https://www.youtube.com/watch?v=5Ey-yrggTGM.
[4] "The new Zelzal-3 ballistic missile launched at Najran forces," al Masirah, August 23, 2016, http://www.almasirah.net/%D9%85%D9%86-%D8%AC%D8%AF%D9%8A%D8%AF-%D8%B5%D8%A7%D8%B1%D9%88%D8%AE-%D8%B2%D9%84%D8%B2%D8%A7%D9%84-3-%D8%A7%D9%84%D8%A8%D8%A7%D9%84%D9%8A%D8%B3%D8%AA%D9%8A-%D9%8A%D8%AF%D9%83-%D9%85%D8%B9%D8%B3%D9%83/; and "Al Houthis announce the launch of a new ballistic missile at Najran," Akhbar Yemen, August 23, 2016, http://www.yemenakhbar.com/yemen-news/463386.html.
[5] Saeed Al Batati, "Yemen army forces battling Al Qaida in last stronghold in Abyan," Gulf News, August 23, 2016 http://gulfnews.com/news/gulf/yemen/yemen-army-forces-battling-al-qaida-in-last-stronghold-in-abyan-1.1883897; and "Security Belt Forces in Abyan Discover Laboratory Manufacturing IEDs in a Court" Aden Press, August 23, 2016 http://www.adenpress.com/index.php/component/content/article/13-news/latest-news/5811-2016-08-22-20-37-50.
[6] "Daily Press Briefing," U.S. Department of State, August 22, 2016, http://www.state.gov/r/pa/prs/dpb/2016/08/261198.htm; and "US announces $146 million for refugee support and return," Goobjoog News, August 23, 2016, http://goobjoog.com/english/?p=32540.
[7] "Spokesman: AMISOM killed 8 militants near Baidoa city," Shabelle News, August 22, 2016, http://www.shabellenews.com/2016/08/spokesman-amisom-killed-8-militants-near-baidoa-city/.
[8] "Kenyan forces deployed to protect telco installations in Mandera County," Goobjoog News, August 23, 2016, http://goobjoog.com/english/?p=32562.


A private garden associated with the house designed in 1882 by R. R. Rowe.
Today the extra strip of garden added by the Keynes family has been reduced by garages and temporary school buildings, but the fruit trees still remain and the acanthus and daylilies by the paths soften the boundary fences. A number of stone fragments were found in the garden which were collected by Mrs. Keynes from the Augustinian Priory at Barnwell.
In 1882 large semi-detached houses were built along Harvey Road by the Victorian architect R.R. Rowe. These houses became the homes to which newly married dons aspired to bring their wives when they were first allowed to marry. Among the first of these academic wives to reside in 6 Harvey Road was Florence Ada Keynes, wife of John Neville Keynes and mother of John Maynard Keynes.
The rear garden of 6 Harvey Road was extended by the Keynes family who gradually acquired a strip of land which lay between their garden and the rear villa gardens along St. Paul's Road. The Ordnance Survey map of 1888 shows this strip of land extending between other properties with access from No. 6 and had a path with several trees planted along its boundary.


You can do this by finding your inner glow. It's there and you can start by making a list of all the things you love in these four areas of your life. What do you love to do or what are you interested in. The number one way to attract love is to make yourself into a vessel that can receive it. A successful relationship comes down to two things: the right person at the right time. The first thing thats important to remember when it comes to relationships is that in general, like attracts like. Find True Love needs tremendous good fortune. One needs not be perfect in order to find that suitable person out there. It is a matter of right place at the right time to meet the right person. Try chanting Nam Myoho Renge Kyo. It might work for some people who really want to find the right person. May 17, 2018 One of the best ways to do this is to start by making friends. It is always said that making friends is the surest way to finding love, and thats because its true; its a great way to build a mutual relationship based on caring and trust. Mar 28, 2007 If there were only one person meant to fall in love with one other person, then only 1 out of every 10, 000 people would find true love during their lifetimes. We all know that this isn't true: people fall in love and have beautiful relationships all the time. Things To Remember When You Feel Like Love Will Never Find You. At the age of 24, I was past his allotted time period where one discovers his or her ability to love.
Commitment does not mean you have to agree with everything, just that you can respect their differences. True love has all three of these components. Having only a couple will show that the relationship has lost its air and needs help. Jul 02, 2014 4 Practical Ways To Find Your Life's Passion And A Career You Love Women@Forbes Files i ForbesWoman OLD DO NOT USE Helping women take their next step forward. Watch video Put the effort in to find someone, but dont act like any breathing body will do. RELATED: How to find lifetime love: 10 secrets from couples married for decades As they reveal how they met. Dating Tips for Finding the Right Person How to Navigate New Relationships and Find Lasting Love. Are you single and looking for love? We find love when we stop looking for it. Its been said a million times, the echo of a field of brokenhearts aching to find the one person who will move mountains to be with them. How Do I Find Love We are one of the most popular online dating sites for men and women. Sign up and start dating, meeting and chatting with other people. How Do I Find Love After years of individual counseling with clients and conducting hundreds of workshops and intensive training programs across the country and around the world, I found that there is only one thing that heals every problem. Being in love with someone brings the kind of love and intimacy that we cannot find in ourselves. It is a closeness that we don't get by loving ourselves, our work, or our other relationships. You might want to explain how being Buddhist and wanting to find love is hypocritical, by the way. They wave at you and you date to come one over. You notice your crush not really enthusiastic about this idea. In private they tell you they would like to leave. Remove from Favorites Add to Favorites. So how does one start to live hisher best life? Some steps to get started: Discover your life purpose. Live in line with it every day. (Ken Soh), and how to attract authentic love into your life.
What I am more interested in is does anyone else feel it is possible to be truly in love with more than one person at the same time? I have always felt alone in this belief. In one of my recent articles on MindBodyGreen, some of the commenters asked me how to go about loving yourself. They especially wanted to know what the specifics are, since it's so easy to tell someone, " Love yourself, " and. AdMake More Meaningful Connections. But one thing todays singles are finding out is that there is one thing in life you cant work for, be good enough for, or accomplish through any persistence of your own. You cant make love happen; it just does. Your work is going to fill a large part of your life, and the only way to be truly satisfied is to do what you believe is great work. And the only way to do great work is to love what you do. If you havent found it yet, keep looking. As with all matters of the heart, youll know when you find it. Meeting the one doesnt always look like a romantic comedy. Your meet likely didnt happen as you were almost hit by a car, and your soontobe lover pushed you out of the way. You probably have never chased after your partner in the pouring rain to stop them from leaving you. Hobbies are simply anything you do thats for fun. You arent getting paid to do it, its something you like to do with your free time that helps you decompress and connect with other people. For a lot of people these days it seems like their only leisure activities are watching television and catching up on Facebook. Take this quiz to find out where you'll be when love finds you! Beauty; Fashion; Where Will You Find Love? May 18, 2010 Advertisement Continue Reading Below. If you feel incomplete and you have this intense need to get out there and find love, ask yourself: What is it that Im trying to find? Chances are, you arent trying to find love. Youre just trying to find yourself, or rather, the love, fulfillment, happiness, fun, or purpose that youre missing on the inside. Should you be looking for your mate in the boardroom, bar, or maybe even while cruising around in your car? Where do you spend your free time? In one of your many scheduled activities. But if they ask me, I'll think about it. K users in the past month Looking for love is a mindset that leads you to believe that love is outside of you. You look out for someone who has your love and is your love. You hope to find them, so that you can find love. However, looking for love isnt about finding another person; its about finding your heart. Keys to Powerful Living: Love By CBN The one who does not love does not know God, for God is love. By this the love of God was manifested in us, that God has sent His only begotten Son into the world so that we might live through Him. In this is love, not that we loved God, but that He loved us and sent His Son to be the propitiation for. Wealthy Healer Retreat; It seems that you really do have to stop looking for love in order to find it. One in particular comes to mind: Do they love me? Figuring out how to tell if someone What Are The Signs Someone Loves You? Little Ways Your Partner Says" I Love You" Without Saying It Live, Give, Love, and Learn: 10 Places to Find Hope By Courtney Carver Find hope in love. I am aware that MS could rob me of health one day, as could many other conditions, but I will not live in fear. Fear and worry may pass by, but they do not have a place in my heart or in my life. Quizzes Society Relationship Love Will I Ever Find Love Will You Find Your Love? Questions By Daringdays Last updated: Aug 3, Do you ever wonder if you will get to find your love? Take up the quiz below and know the chances of that happening. Do you think that one of your best friends. How to Find Friends and Fight Loneliness After 60 By but as I get older, it is becoming easier to find people to do things with and one does not need intimate friends or partners, although if you find them it is very nice. Harder would love to meet some women my own age to talk to I have two wonderful friends one moved to Alberta sad. EXPLORE GOD'S LOVE Where do I look to find God in this world of tragedy and pain? Tragedy and pain are disorienting. We dont want to experience either, yet life keeps bringing them on. Its message speaks of helping those one doesn't know, maybe those with whom one has never spoken, those who are different from. How Do You Find True Love We are one of the most popular online dating sites for men and women. Sign up and start dating, meeting and chatting with other people. How Do You Find True Love asian date sites speed dating. Strange Questions That Help You Find Your Life Purpose September 18, 2014 July 8, 2018 14 minute read by Mark Manson O ne day, when my brother was 18, he waltzed into the living room and proudly announced to my mother and me that one day he was going to be a senator. QuizHow to Know if You're Really in Love It is a very common question, " How can I tell I'm in love? What feels like love to one person may be nothing more than attraction to another. Heres How To Make Someone Fall In Love With You. When researchers asked people to tell the stories of how they fell in love, what were the eleven most common factors. At the end of the day, you have two choices in love one is to accept someone just as they are and the other is to walk away. We owe it to ourselves to live the greatest life that were capable of living, even if that means that we have to be alone for a very long time. Read This If Youre Worried That Youll Never Find The One. They had a blast and got married one year later. Time is on your side Remember what the Garter Brides always say: Its never too late to find true love How Do I Find Love We are one of the most popular online dating sites for men and women. Sign up and start dating, meeting and chatting with other people. How Do I Find Love You will never find or experience the true love you are looking for in this world alone. Each of us and the world around us is too soaked in sin. The great news is that there is One who is the very definition of Love and you can be in relationship with Him! There is Only One Source of True Love. Let us return to Leahs story for a moment. Love is very hard to find, you dont want to go out and look for it, just live and let it come to you. The harder you try, the more evasive love. Could you find love while walking your dog?


For the longest time, the ability to just talk to a machine was a pipedream, the stuff of sci-fi. No longer.
Over the years, our interaction with machines has evolved — from command line prompts to mouse-based, graphical UIs to touch-screen interfaces. And while tech keeps improving, it still requires us to adapt to new forms of communication. Enter voice marketing.
We can trace efforts to build voice technology back to 1960 with IBM's Shoebox, the first attempt at voice recognition.
But it is only in the past decade, thanks to the advances in Artificial Intelligence (AI) and Natural Language Processing (NLP), that conversational interfaces have become within reach of the general population. We've seen an explosion in AI-powered voice assistants and chatbots — from smartphones to smart speakers — that now let us converse with our computers, phones, and devices.
Voice was first introduced to mass market through Apple's Siri, followed by Google Now. Since then, voice usage on mobile has exploded. Siri and Google Now lead the pack when it comes to voice assistants on smartphones; Amazon's Echo and Google Home are the leaders in the smart speaker category. Voice has crept into people's lives and integrated into their daily routine.
So, when and how are people using voice?
While the trend started with voice assistants on smartphones, the trigger that turns voice into a product phenomenon could be smart speakers. With Amazon's Echo leading the way, sales of smart speakers have risen exponentially in the past few years.
The evidence is around us. Nearly 19 million US households now use smart speakers, a 50 percent increase from December 2017 to February 2018. At the same time, the number of Alexa skills has seen a 3x increase year-over-year, indicating exploding interest in the developer community to engage users through Alexa devices.
At home, with smart speakers generally situated in living rooms or bedrooms, it's no surprise they're being used to handle daily household tasks and activities like listening to music, checking forecasts, hearing the news, etc.
Interestingly, research shows that smart speaker users of voice assistants are more engaged than mobile phone users. Smart speakers are changing user behavior, conditioning us to use voice for more activities and making it more normal. This could in turn boost usage of voice on smartphones and other devices as customers grow accustomed to using voice interfaces for daily tasks.
It is quite clear that voice interfaces are here to stay. Next, we need to understand the implications of this new phenomenon.
In his 2016 Google I/O keynote, Sundar Pichai, Google's CEO, announced that 20 percent of all mobile searches on Android are voice queries. According to Location World, more than 40 percent of adults used voice-based search on a daily basis in 2016. And predictions by ComScore estimate that more than 50 percent of searches will be voice-based by 2020.
Why this sudden jump in adoption? Increasingly sophisticated technology has made voice search less error-prone and able to decipher more complexity in queries, making voice more efficient and valuable to users.
So what are the implications of this impending revolution? Traditional search has conditioned us to be minimalistic in using search terms. But as voice assistants become capable of handling increasingly complex queries, our queries and interactions with the assistants will become longer and more conversational. This can provide more context about user intent, which, in turn, can help marketers better assess where the user is along their customer journey.
As voice assistants become more integrated into our daily lives, they are going to become an increasingly important channel through which we discover information, make purchases, and interact with websites.
The nature of voice search results means there will be intense pressure to capture the top spot in voice search engine results. If customers use smart speakers to save time, they will be more likely to go with the choice presented by their smart speaker, rather than ask for more options.
This will have a long-term effect on consumer behavior as shoppers shift their allegiance from brands to their voice assistant platforms.
All this brings us to a larger and more consequential long-term implication of voice assistants: The consumer belief in their voice assistants as a trusted source of truth. As voice platforms become the default interfaces for home systems, appliances, and the internet, consumer trust in them to deliver the best possible choice will keep growing, possibly displacing brand loyalty.
That's why brands need to start thinking about their voice strategy now. As the use of voice assistants becomes more prevalent, marketers need to assess their strategy to ensure they are ready to adapt to the changing needs and behaviors of consumers.
First, voice search queries are more specific (and local) than your regular search, so your website content should be updated accordingly.
Second, when considering your strategy for voice interfaces, less is always more. Voice-based engagement should be as non-intrusive as possible — it's easy to turn off customers with messages that scream advertising. Your voice engagement's primary focus should be to engage and provide value to the user.
Take a look at brands like Moet Hennessy, Tide, and Patron. They have all built Alexa skills so they can engage their users even as they educate or inform them. Brands can create loyal customers — focusing on content — without having to overtly market to them.
Patron's "Ask Patron" skill on Alexa enables users to ask for cocktail recommendations, recipes, and tips. This is part of Patron's effort to create a more meaningful and direct relationship with their users by creating experiences across their user journey. Since Patron added its Ask Patron skill on Alexa, it has accumulated 36,000 users of the skill, the majority of whom are repeat customers indicating high engagement.
That's voice marketing engagement worth shouting from the rooftops.
You don't have to build a voice marketing strategy from scratch. The lessons used for mobile apps can be applied to voice as well. Marketers ought to use the additional nuance and context provided by these conversational interfaces to ensure a more personalized and one-to-one engagement strategy with their users.
As voice interfaces look to fundamentally transform how we interact with our personal technologies, marketers need to understand this disruptive force to ensure they brands can maintain and grow their relationships with their customers.
Leanplum is a mobile engagement platform that helps forward-looking brands like Grab, Tinder, and Tesco meet the real-time needs of their customers. Schedule your personalized demo here.


House of Commons Debates, First Session, Twenty-Fifth Parliament, Volumes I, II, and III (2018). 10.5" x 7" x 15.5". Photo by Cory Ransom.
This work consists of a series of three hardcover bound volumes that were created using reproductions of the 1962 Canadian Federal Parliamentary Hansards (25th Parliament) —a time when the debate was squarely focused on the Cuban Missile Crisis. From each of the three volumes, phrase that have resonance with today's political discourse and landscape were selected and laser cut in a cascading fashion throughout each volume. Each of the 3,463 pages has been individually laser cut before being bound.
House of Commons Debates, First Session, Twenty-Fifth Parliament, Volumes III (2018). Photo by Cory Ransom.
House of Commons Debates, First Session, Twenty-Fifth Parliament, Volumes II (2018). Photo by Cory Ransom.
Hardcover box, 3,463 pages, un-bound.
Similar to the 1st Edition, phrases have been cut into reproductions of the 1962 Canadian Federal Parliamentary Hansards (25th Parliament), however they have not been bound into volumes, but rather are left unbound for public viewing.
House of Commons Debates, First Session, Twenty-Fifth Parliament, Volumes I, II, and III (2018). Photo by Yael Eban.
House of Commons Debates, First Session, for public viewing (2018), the Banff Centre for Arts and Creativity, March 2018. Photo by Alex Farko.


New Price! This gorgeous home is nestled in the cozy, gated community of Cherry Hill. This home has elegant finishes with hard wood floors, granite countertops, an island, a breakfast bar, pot filler above the stove, spice cabinet, and office space off the kitchen. No detail was spared! Large backyard with a beautiful pergola, that's perfect for entertaining. Large bedrooms! Great size master bedroom; tray ceilings with a great closet space! Bonus room area can be additional living space!


Carin "CJ" Evans is a Texas native. She graduated from Regent University School of Law in Virginia Beach, Virginia in 2001. Upon graduation she returned to Texas and began practicing in the Rio Grande Valley.


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Leia malleolus är en tvåvingeart som beskrevs av Freeman 1954. Leia malleolus ingår i släktet Leia och familjen svampmyggor. Inga underarter finns listade i Catalogue of Life.

Källor

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redpajama_set_name: RedPajamaWikipedia


Chapter 17.106 of the Portland City Code shall be known as the Deconstruction of Buildings Law.
D. Minimize the adverse impacts associated with building removal.
A. "Certified Deconstruction Contractor" means a contractor licensed with the Oregon Construction Contractors Board (CCB) that has successfully completed a deconstruction certification program recognized by the Bureau of Planning and Sustainability. A firm will be considered certified if at least one person currently employed by the firm is certified.
B. "Deconstruction" means the systematic dismantling of a structure, typically in the opposite order it was constructed, in order to maximize the salvage of materials for reuse, in preference over salvaging materials for recycling, energy recovery, or sending the materials to the landfill.
C. "Director" means, the Director of the Bureau of Planning and Sustainability or his or her authorized designee.
D. "Primary Dwelling Structure" means one and two-family structures (detached and attached) based on current permitted occupancy at the time of demolition permit application. Primary Dwelling Structures do not include accessory structures such as garages or accessory dwelling units.
E. "Recycling" means the processing of waste materials into new products or material feed stock for products. Materials that can be recycled include but are not limited to concrete, metal piping, and asphalt roofing shingles.
F. "Responsible Party" means any owner or person in control of a primary dwelling structure, or their authorized agent.
G. "Reuse" means the utilization of a product or material that was previously installed for the same or similar function to extend its life cycle. Materials salvageable for reuse include but are not limited to cabinets, doors, hardware, fixtures, flooring, siding, and framing lumber.
1. Any rule adopted pursuant to this Section shall require a public review process. Not less than 10 nor more than 30 days before such public review process, notice shall be given by publication in a newspaper of general circulation. Such notice shall include the place, time and purpose of the public review process and the location at which copies of the full set of the proposed rules may be obtained.
2. During the public review, the Director shall hear testimony or receive written comment concerning the proposed rules. The Director shall review the recommendations, taking into consideration the comments received during the public review process, and shall either adopt the proposed rules, modify or reject them. Unless otherwise stated, all rules shall be effective upon adoption by the Director and shall be filed in the Office of the Director and with the City Auditor's Portland Policy Documents repository.
C. The Director may temporarily suspend or modify the requirements of this Chapter based on a determination that such requirements are temporarily infeasible due to economic or technical circumstances. The Director's determination to temporarily suspend or modify shall be filed as a report with the City Council. The Director's determination shall be effective after the Council has accepted the report.
A. Whenever the responsible party has been given a written notice or order pursuant to this Chapter or has been directed to make any correction, pay a penalty or to perform any act and the responsible party believes the finding of the notice or order was in error, the responsible party may have the notice or order reviewed by the Director. If a review is sought, the responsible party will submit a written request to the Director within 10 days of the date of the notice or order. Such review will be conducted by the Director. The responsible party requesting such review will be given the opportunity to present evidence to the Director. Following a review, the Director will issue a written determination. Nothing in this Section shall limit the authority of the Director to initiate a code enforcement proceeding under Title 22.
B. A responsible party may appeal the Director's written determination to the Code Hearings Officer in accordance with Portland City Code Chapter 22.10. The filing of an appeal request will remain the effective date of a penalty until the appeal is determined by the Code Hearings Officer. If, pursuant to said appeal hearing, payment of a penalty is ordered, such payment must be received by the Director or postmarked within 15 calendar days after the order becomes final.


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We hope you can find what you need here. We always effort to show a picture with HD resolution or at least with perfect images. Kohler Undermount Kitchen Sink Inspirational Undermount Kitchen Sinks Kitchen Sinks the Home Depot can be beneficial inspiration for those who seek an image according specific categories; you can find it in this site. Finally all pictures we have been displayed in this site will inspire you all..


JOHN SUTTON double-checked with his old man about Mark McGhee's pedigree as a player - and was left in no doubt he will benefit from the Motherwell gaffer's words of wisdom.
New Fir Park striker Sutton is quick to praise the likes of Andy Millen at St Mirren and former Wycombe boss Paul Lambert for helping him improve as a player over the past two years.
And he's certain that McGhee - as a former hitman himself - will help take his game to a new level.
Sutton - at only 24 - can be forgiven for not being completely up to speed on McGhee's stats. After all, McGhee won the Cup Winners' Cup with Aberdeen the year before he was born!
However after doing his homework on the former Aberdeen, Hamburg, Celtic and Scotland striker he's excited about the new season ahead at Fir Park and a possible debut on Saturday against Dundee United.
Sutton said: "I knew that the manager had played for big clubs and was no mean striker himself.
"I asked my dad about him and he was well aware of his qualities and I must admit I'm looking forward to having a former striker as a manager now.
"At Saints Andy and Mark Reilly were really helpful to me and obviously one of the reasons I went to Wycombe last season was the fact that Paul Lambert was so keen to sign me.


where do I install my API key ?
I've downloaded the dashboard but can't install it?
Have you installed it in your WordPress install?
Once you have uploaded and activated the WPMU DEV Dashboard plugin (just like any other WordPress plugin), all you need to do is log in with the same username and password that you use to log into this site.
Our API will recognize your login credentials and automatically set things up for you.
To enable automatic updates & one-click installation of our products, and direct support, through the Dashboard plugin, you will also need to register your site.
To do so, click the link in the alert that appears as soon as you have logged in (screenshot).
Is it possible I need to be a .org blog instead of a .com to see plugins??