Type I glycogen storage disease

Type I glycogen storage disease

Simplified scheme of glycogen synthesis and degradation in the liver. Key to scheme: UDP-glucose, uridine diphosphoglucose; 1. hexokinase/glucokinase; 2. glucose 6-phosphatase; 3. phosphoglucomutase; 4. glycogen synthase; 5. branching enzyme; 6. glycogen phosphorylase; 7. debranching enzyme; 8. phosphofructokinase; 9. fructose 1,6-bisphosphatase; 10. acid maltase; 11. pyruvate dehydrogenase

Created by Dr Joseph I. Wolfsdorf; used with permission

See this image in context in the following section/s:

• Etiology