GLYCOGEN METABOLISM Flashcards
What are the rate limiting enzymes in glycogen metabolism ?
Glycogen Synthase
Glycogen phosphorylase
What is the branching enzyme in glycogen metabolism ?
(4:6 transferase)
What is the Debranching enzyme
4:4 transferase
Glucose 1 phosphate + UTP is converted to what ?
UDP GLUCOSE
What is the glycogen primer in glycon synthesis ?
Glycogenin
What bond does glycogen phosphorylase break ?
Alpha (1,4) glycosidic bond between glycogen primer and UDP glucose
Which Debra ching enzyme removes the residual 3 glucose in glycogenlysis ?
4:4 transferase
Which enzyme hydrolysed the glucose residue at the former 1,6 branch in glycogenlysis ?
Alpha 1,6 glycosidase
What enzymes are activated by phosphorylation by glucagon in Glycogenlysis ?
CAMP
Protien kinase A
Glycogen phosphorylase kinase
Glycogen phosphorylase
Increases glycogen breakdown to glucose
Which enzymes are activated by dephosphorylation by insulin in glycogenis?
Tyrosine kinase A
Protien Phosphatase
Glycogen Synthase
Increases glycogen storage
What pathway does epinephrine activate during stress in muscle ?
Alpha 1 receptor
Increases Ca2+ through ER
Activates glycogen phosphorylase kinase
Activates glycogenlysis
What are the common symptoms of glycogen storage disease ?
Severe Hypoglycemia
Hepatomegaly
Muscle cramps
What enzyme does the most common glycogen storage disease affect and what are the symptoms and CF ?
GLUCOSE 6 POSPHATE
(VAN GIERKE)
Increased Glycogen in liver (GOUT)
AR
PROTRUDING ABDOMEN
3 to 6 months
GROWTH FAILURE
Lactic acidosis
What is the deficiency in Pompeo disease ?
Lysosomal 1,4 glycosidase (Acid Maltase)
What enzyme does Cori disease affect ?
Debranching enzyme
Alpha 1,6 glycosidase
4 alpha D glucanotransferase
Treatment : High protein diet
