Glycogen, Gluconeogenesis, and HMP shunt Flashcards
Hers disease deficient in
Hepatic glycogen phosphorylase
What is the rate limiting enzyme in glycogenolysis
Glycogen phosphorylase
Purpose of Malate shuttle in Gluconeogenesis
Transports OAA out of mitochondria into cytoplasm
Von Gierke clinical
- Severe hypoglycemia
- Lactic acidosis
- Hepatomegaly
- hyperlipidemia
- Hyperuricemia
- Short, doll-like face
Glycogen phosphorylase is stimulated by
Glucagon
Epinepherine
AMP
PEP carboxykinase converts
OAA to PEP
Cori disease deficient in
Glycogen debranching enzyme
McArdle disease deficient in
Muscle glycogen phosphorylase
Glucose-6-phosphatase converts
Glucose 6-P to Glucose
Andersen (amylopectinosis) disease deficient in
Branching enzyme
G6PD genetics
x-linked recessive
Glucose 6-phosphatase converts
Glucose 6-P to Glucose
Andersen clinical
Infantile hypotonia
Cirrhosis
What are the 2 major functions of HMP shunt
NADPH production
Source of ribose 5-phosphate for nucleotide synthesis
Pyruvate Carboxylase converts
Pyruvate to OAA
McArdle clinical
Muscle cramps
Weakness on exercise
Myoglobinuria
High NADH due to alcohol favors formation of
Lactate from Pyruvate
Malate from OAA
Glycerol 3-phosphate from DHAP
Glycogen synthase converts
UDP-Glucose to Glycogen
Pyruvate carboxylase coenzyme
Biotin
Cori clinical
Mild Hypoglycemia
Liver enlargement
Pompe clinical
Cardiomegaly
Muscle weakness
Glycogen phosphorylase converts
Glycogen to Glucose 1-P
Pompe disease deficient in
Lysosomal α1,4-glucosidase
Fructose-1,6-bisphosphatase converts
Fructose 1,6-BP to Fructose 6-P
