Page 255 - Color Atlas Of Pathophysiology (S Silbernagl Et Al, Thieme 2000)

A. Causes of Glycogen Storage Diseases I–VIII and Fructose Intolerance
Lysosomal breakdown Enzyme defects
Glycogen
Enzymes esp. in liver
Enzymes esp. in liver
II Enzymes esp. in skeletal
Lysosomal VIII muscles
α-glucosidase Enzymes in many organs
Phosphorylase-
kinase IV
VI Fructose Branching enzyme +
Glycogensynthetase
Phosphorylase
V
Fructose-1-P
Fructose- UDP-glucose
1-P-aldolase Lactate Carbohydratis, Lipidoses
Limit dextrin
Fructose
intolerance
III Trioses Pyruvate Citrate
Debranching cycle
enzyme
Glucose-1-P ATP
Glucose Fructose-1,6-P 2 - Plate 8.2
Glucose-6-P aldolase
VII
Ia Phospho-
Glucose-6- fructokinase Fructose-1,6-P 2
phosphatase Fructose-6-P
B. Effects of Glycogen Storage Diseases
I, III,
Glycogen Other VI, VIII
organs Liver types
Glucose
Liver Blood glucose
Hepatomegaly

Glucose ATP
Muscle
Glycogen Hypoglycemia
II,IV
V, VII
Other types Muscle types
During severe muscle activity:
Cardiomegaly, Cramps, weakness
muscle weakness
(respiratory muscles!)
Myoglobinuria
ATP
Cardiorespiratory failure Renal failure 245

Silbernagl/Lang, Color Atlas of Pathophysiology © 2000 Thieme
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