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Glycogen Storage Diseases
Terms in this set (6)
Type I: Von Gierke Disease
Hypoglycemia occurs from inability of liver to generate glucose either by glycogenolysis or gluconeogenesis
Type II: Pompe Disease
Lysosomal α 1-4 Glucosidase Defect
There is accumulation of normal glycogen in cytoplasmic vacuoles
Broad effects, primarily liver, heart and muscle
Normal levels of blood glucose are observed.
Type III: Cori's Disease
Debranching Enzyme Defect
Glycogen increased, but deposits have short outer branches
Type IV: Andersen's disease
Branching Enzyme Defect
Normal amount of glycogen with long outer branches
Type V: McArdle Disease
Skeletal Muscle Glycogen Phosphorylase Defect
Skeletal muscle is affected, but the liver is normal.
Muscle shows a somewhat higher level of normal glycogen.
Weakness and cramping of skeletal muscle upon exercise.
There is no rise in blood lactate upon strenuous exercise.
Myoglobinemia and myoglobinuria
Type VI: : Hers Disease
Liver Glycogen Phosphorylase Defect
Liver is affected, but skeletal muscle is normal.
Following glucagon, there is no increase in blood glucose.
Hypoglycemia but not as severe as Type I
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