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Glycogen Metabolism Reading
UC MED 2015
33
Medical
Graduate
10/23/2011

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Term
"R"
Definition
The relaxed (active) form of phosporylase
Term
"T"
Definition
the tense (inactive) form of phosphorylase
Term
PK
Definition
Pyruvate Kinase
Term
PFK-1
Definition
phosphofructokinase-1
Term
PFK-2
Definition
phosphofructokinase-2
Term
F1,6BP
Definition
fructose-1,6-bisphosphate
Term
Protein kinase A
Definition
the same as the cAMP dependent protein kinase
Term
G1P
Definition
glucose-1-phosphate
Term
G6P
Definition
glucose-6-phosphate
Term
a(1,4)
Definition
an alpha linkage between two sugars, one at carbon 1, the other at carbon 4
Term
a(1,6)
Definition
an alpha linkage between two sugars, one at carbon 1, the other at carbon 6
Term
UDPG
Definition
UDP-glucose
Term
The largest glycogen stores are in the?
Definition
liver and muscle
Term
What removes glucosyl units one at a time from the ends of the glycogen chains,converting them to
glucose 1-phosphate without resynthesizing UDP-glucose or UTP?
Definition
glycogen phosphorylase
Term
What removes the glucosyl residues near each branch point?
Definition
debranching enzyme
Term
Glucagon activates which two pathways?
Definition
Gluconeogenesis and Glycolysis
Term
G6P is converted to G1P by?
Definition
phosphoglucomutase
Term
What is the enzyme that attaches the glucosyl residues in 1,4-bonds?
Definition
glycogen synthase
Term
When the chain reaches approximately 11 residues in length, a 6- to 8-residue piece is cleaved by ___________ and reattached toa glucosyl unit by an
alpha-1,6-bond.
Definition
amylo-4,6-transferase
Term
What is the protein to which glycogen is attached, glycosylates itself (autoglycosylation) by attaching the glucosyl residue of UDP-glucose to the hydroxyl side chain of a serine residue in the protein?
Definition
glycogenin
Term
McCardle Disease
Definition
genetic deficiency in muscle glycogen phosphorylase (inconvenience)
Term
Hers disease
Definition
genetic deficiency in liver glycogen phosphorylase (lethal)
Term
What can hydrolyze glycogen in a lysosome?
Definition
lysosomal glucosidase
Term
Pompes disease
Definition
genetic defect of lysosomal glucosidase (children with this normally die of heart disease)
Term
Glucagon causes a...
Definition
a cAMP directed phosphorylation cascade
Term
What happens to glycogen phosphorylase when glucagon is present?
Definition
It is phosphorylated(by Protein Kinase A plus 9 more) and goes from the b(inactive) to a(active) form.
Term
What happens to glycogen synthase when glucagon is present?
Definition
It is phosphorylated(by Protein Kinase A), it goes from the a(active form to the D or b(inactive) form
Term
What class of proteins remove the phosphate groups,bound to serine or other residues of enzymes,by hydrolysis?
Definition
Protein phosphatases
Term
What protein is one of the major protein phosphatases involved in glycogen metabolism, removes phosphate groups from phosphorylase kinase, glycogen phosphorylase, and glycogen synthase?
Definition
Hepatic Protein Phosphatase-1 (HPP-I)
Term
What is as an allosteric effector, inhibits liver glycogen phosphorylase a by stimulating dephosphorylation of this enzyme?
Definition
glucose
Term
Epinephrine has two receptors which are?
Definition
alpha and beta agonist receptors
Term
What epinephrine receptor transmits a signal through G proteins to adenylate cyclase increasing cAMP, thus activating protein kinase A. (This part of epinephrine is similar to glucagon)?
Definition
epinephrine binding to beta-agonist receptor
Term
Epinephrine binding at this receptor in the hepatocyte activates glycogenolysis and inhibits glycolysis by increasing levels of Ca2+?
Definition
epinephrine binding to alpha-receptors
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