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Glycogen Metabolism and Control
EXAM 2
60
Medical
Professional
08/30/2010

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Term
why not store excess glucose as fat instead of glycogen?
Definition
fat cannot be mobilized very rapidly

it cant be used as a source of energy i the absence of oxygen.

fat cant maintain glucose levels needed to feed the brain.
Term
why not store glucose as free glucose?
Definition
takes too much energy to push into a cell.

cause water gradient to flow into cell causing lysis
Term
what makes glycogen more water soluble?
Definition
branching
Term
what enzyme initiates glycogen breakdown?
Definition
glycogen phosphorylase
Term
what is the name of two enzymes of the debranched glucose?
Definition
4 alpha glucanotransferase and amylo alpha 1,6 glucosidase
Term
what is the function of 4 alpha glucanotransferase?
Definition
removes a strand of three glucosyl residues from a four-glucosyl residue branch of the glycogen molecule
Term
what is the function of amylo-alpha glucosidase?
Definition
hydrolyzes the alpha 1-6 linkage of the single glucosyl residue
Term
what is glycogen phosphorylase specific for?
Definition
a-1,4 glycosidic linkages
Term
on what end does glycogen phosphorylase act on
Definition
non reducing end of glycogen until 4 residues away from a,1-6
Term
what allows the enzyme phosphorylation to continue degradation of glycogen
Definition
debranching
Term
what inactivates glycogen phosphorylase
Definition
dephosphorylation
Term
explain the regulation of glycogen phosphorylase
Definition
phosphorylase kinase phosphorylates glycogen phosphorylase to the a form

phosphoprotein phosphatase dephosphorylates glycogen phosphorylase to the inactive form
Term
what is the effect of glucagon and epinephrine on glycogen phosphorylase?
Definition
activation of glycogen phosphorylase
Term
what is the effect of insulin on glycogen phosphorylase
Definition
de phosphorylation
Term
what effect does PKA play on phosphorylase activity?
Definition
it activates it to its active form
Term
what happens to PKA when cAMP is increased
Definition
it will increase
Term
what is the function of the calmodulin complex on the phosphorylase kinase?
Definition
it activates both the a and b forms of phosphorylase kinase
Term
what are the effects of AMP on phosphorylase b?
Definition
activation
Term
what is the function of glucose and ATP on phosphorylase a?
Definition
inhibition
Term
what is the fate of degraded glycogen in liver?
Definition
G6P is cleaved by glucose 6 phosphatase to glucose to be released into the circulation
Term
what is the fate of glycogen in the peripheral tissues?
Definition
G6P will undergo glycolysis
Term
what is the fate of glycogen in white muscle?
Definition
lactate
Term
what is the fate of glycogen in red muscle
Definition
pyruvate, acetyl coA, and ultimately CO2
Term
where is teh enzyme glucose 6 phosphatase located?
Definition
liver and kidney
Term
how is glucose released into the blood?
Definition
a bidirectional facilitated glucose transporter (glut 1) at the plasma membrane
Term
what is the name of the activated glucose from which glycogen can be synthesized?
Definition
UDP glucose
Term
what makes UDP-glucose energetically favorable?
Definition
hydrolysis of inorganic pyrophosphate by pyrophosphatase
Term
what is the immediate donor of glucose residues to be added at the non reducing end of glycogen
Definition
UDP glucose
Term
what places glucose at the non reducing end of glycogen
Definition
glycogen synthase
Term
this provides a primer for glycogen synthesis by glycogen synthase
Definition
glycogenin
Term
what effect does phosphorylation have on glycogen phosphorylase and glycogen synthase?
Definition
activates glycogen phosphorylase

inhibits glycogen synthase
Term
what form is glycogen synthase in when dephosphorylation occurs
Definition
active form a
Term
when are liver glycogen levels high?
Definition
shortly after a meal
Term
how long does stored glycogen last?
Definition
12-24 hours
Term
what does muscle do with free glucose?
Definition
catabolize it
Term
is muscle glycogen important for maintenance of blood glucose in the post-absorbtive state
Definition
No
Term
is muscle glycogen important for maintenance of blood glucose in the post-absorbtive state
Definition
No
Term
what is the relationship between phoshphorylase a, glycogen synthase, and phosphoprotein phosphatase
Definition
phoshporylase a inhibits dephosphorylation of glycogen synthase b by phosphoprotein phosphatase
Term
w
Definition
Term
what is the relationship b/t phosphorylase and phosphoprotein phosphatase
Definition
phosphoprotein converts the phosphorylase to the inactive b form
Term
what is the relationship between phosphoprotein phosphatase and glycogen synthase
Definition
the active form of phosphoprotein phosphatase activates glycogen synthase
Term
what is the relationship between phosphoprotein phosphatase and glycogen synthase
Definition
the active form of phosphoprotein phosphatase activates glycogen synthase
Term
what is the relationship between glucagon and epinephrine in reference to glycogenolysis?
Definition
It stimulates it by increasing intracellular cAMP
Term
describe the signaling process of increased cAMP.
Definition
activates PKA
PKA activates phosphorylase kinase
Phosphorylase kinase phosphorylates and activates glycogen phosphorylase
phosphorylase kinase phosphorylates and inhibits glycogen synthase
Term
when epinephrine binds in the liver, what is the effect?
Definition
stimulation of glycogenolysis by generation of inositol 1,4,5 triphosphate and diaglycerol
Term
how does increased cytosolic Ca effect signaling?
Definition
this activates phosphorylase kinase which activates glycogen phosphorylase with glycogenolysis.
Term
how does epinephrine affect signaling in the heart and skeletal muscle?
Definition
cAMP-->PKA-->phos. of phosphorylase kinase-->which phos. and activates glycogen phosphorylase.
Term
what activates muscle contraction?
Definition
intracellular Ca is increased and that activates phosphorylase kinase and glycogen phosphorylase
Term
what kind of transporter does hepatocytes have?
Definition
glut2 transporter
Term
what does glut2 transporter allow?
Definition
uptake of glucose to be dependent on the glucose concentration in the blood.
Term
how does insulin stimulate glycogen synthesis
Definition
activating phosphoprotein phosphotase which in turn dephosphorylates glycogen synthase.
Term
what does insulin induce in the muscle on its membrane?>
Definition
glut4 transporter-->glycogenesis
Term
what are the three types of liver glycogen storage diseases?
Definition
von gierke's disease, limit dextrinosis, amylopectinosis
Term
what is the enzyme deficiency in Von Gierke's disease?
Definition
glucose 6 phosphatase
Term
what is the enzyme deficiency in limit dextrinosis?
Definition
debranching enzymes
Term
what is the enzyme deficiency in amylopectinosis?
Definition
Branching enzyme
Term
what are some laboratory findings of Von Gierke
Definition
administration of glucagon or epinephrine increases blood lactate with little or no rise in blood glucose

hyperlipidemia, hyperuricemia.
Term
what are the name of the glycogen storage diseases that affect the muscle?
Definition
McArdle's syndrome and Pompe's disease
Term
what is the enzyme deficiency in McArdle's syndrome
Definition
muscle phosphorylase
Term
what are some clinical manifestations of McArdle's syndrome
Definition
exercise intolerance, intense myoglobinuria
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