Study Set Content:
Conversion of G6P to F6P
Phosphoglucose isomerase (PI)
Cleavage of FBP to DHAP and GLAP/G3P
Aldolase
Isomerization of DHAP to a second molecule of GLAP/G3P
Triose phosphate isomerase (TIM)
Dehydrogenation and phosphorylation of GLAP/G3P to 1,3- bisphosphoglycerate
Glyceraldehyde-3- phosphate dehydrogenase
Transfer of 1-phosphate group from 1,3- bisphosphoglycerate to ADP which yields ATP and 3-phosphoglycerate
Phosphoglycerate kinase (PGK)
Isomerization of 3- phosphoglycerate to 2- phosphoglycerate
Phosphoglycerate mutase (PGM)
Dehydration of 2- phosphoglycerate to PEP
Enolase
Transfer of phosphate group from PEP to ADP to yield second molecule of ATP and pyruvate
Pyruvate kinase (PK)
G6P
glucose-6-phopshate
F6P
fructose-6-phosphate
FBP
- fructose-1,6-bisphosphate
DHAP
dihydroxyacetone phosphate
• GLAP/G3P
glyceraldehyde-3-phosphate
PEP
phosphoenolpyruvate
ADP
adenosine diphosphate
ATP
adenosine triphosphate
are inherited inborn errors of carbohydrate metabolism
Glycogen storage diseases
In general, they occur due to a lack of specific enzymes involved in the breakdown of
glycogen
In general, they occur due to a lack of specific enzymes involved in the breakdown of glycogen and result in an abnormal buildup of glycogen in the
liver or skeletal muscles.
The inability to mobilize glucose from glycogen results in
hypoglycemia
