GLYCOGEN STORAGE DISEASES Flashcards
a self-glucosylating protein which has a catalytic activity;
reactive site is the OH group of tyrosine residues – forms an O
glycosidic linkage. STIMULUS: depletion of glycogen
Glycogenin
non-reducing end = Carbon 4
α-1,4 glycosidic bond:
Anabolic hormone
Promotes the storage of glucose into glycogen
Also promotes converting glucose intro triacylglycerides and the
storage of triacylglycerides in the adipose tissue
Insulin
Acts to maintain glycose availability in the absence of dietary glucose
by stimulating the release of glucose from the liver
Stimulates glycogenolysis and gluconeogenesis
Glucagon
Is another hormone that is released in response of the central
nervous system once it is under stress
Epinephrine
enzyme deficiency of Type 0
glycogen synthase
enzyme deficiency of Von Gierke’s disease
Type IA
glucose 6-phosphatase
enzyme deficient of of Pompe’s disease type 2
lysosomal alpha 1
glucosidase (acid maltase)
enzyme deficient of Cori’s disease
Type 3
liver/muscle debranching enzyme
enzyme deficient in type 4 Andersen disease (amylopectinosis)
branching enzyme
enzyme deficient in type 5 McArdle’s disease (Myophosphorylase deficiency)
Muscle Phosphorylase
type 6 Hers disease
enzyme deficient
liver glycogen phosphorylase
type 7 tarui’s disease
phosphofructokinase
liver and erythrocyte phosphofructokinase 1
glycogen granules can be engulfed by _________ where acid maltase catalyzes hydrolysis of glycogen to glucose
lysosomes
in ______ but not muscle glucose 6-phosphatase catalyzes hydrolysis of glucose 6-phosphate yielding glucose that is exported
liver
