Term
why not store excess glucose as fat instead of glycogen? |
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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. |
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Term
why not store glucose as free glucose? |
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Definition
takes too much energy to push into a cell.
cause water gradient to flow into cell causing lysis |
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Term
what makes glycogen more water soluble? |
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Definition
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Term
what enzyme initiates glycogen breakdown? |
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Definition
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Term
what is the name of two enzymes of the debranched glucose? |
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Definition
4 alpha glucanotransferase and amylo alpha 1,6 glucosidase |
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Term
what is the function of 4 alpha glucanotransferase? |
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Definition
removes a strand of three glucosyl residues from a four-glucosyl residue branch of the glycogen molecule |
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Term
what is the function of amylo-alpha glucosidase? |
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Definition
hydrolyzes the alpha 1-6 linkage of the single glucosyl residue |
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Term
what is glycogen phosphorylase specific for? |
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Definition
a-1,4 glycosidic linkages |
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Term
on what end does glycogen phosphorylase act on |
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Definition
non reducing end of glycogen until 4 residues away from a,1-6 |
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Term
what allows the enzyme phosphorylation to continue degradation of glycogen |
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Definition
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Term
what inactivates glycogen phosphorylase |
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Definition
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Term
explain the regulation of glycogen phosphorylase |
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Definition
phosphorylase kinase phosphorylates glycogen phosphorylase to the a form
phosphoprotein phosphatase dephosphorylates glycogen phosphorylase to the inactive form |
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Term
what is the effect of glucagon and epinephrine on glycogen phosphorylase? |
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Definition
activation of glycogen phosphorylase |
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|
Term
what is the effect of insulin on glycogen phosphorylase |
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Definition
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|
Term
what effect does PKA play on phosphorylase activity? |
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Definition
it activates it to its active form |
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Term
what happens to PKA when cAMP is increased |
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Definition
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Term
what is the function of the calmodulin complex on the phosphorylase kinase? |
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Definition
it activates both the a and b forms of phosphorylase kinase |
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Term
what are the effects of AMP on phosphorylase b? |
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Definition
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Term
what is the function of glucose and ATP on phosphorylase a? |
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Definition
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|
Term
what is the fate of degraded glycogen in liver? |
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Definition
G6P is cleaved by glucose 6 phosphatase to glucose to be released into the circulation |
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Term
what is the fate of glycogen in the peripheral tissues? |
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Definition
G6P will undergo glycolysis |
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|
Term
what is the fate of glycogen in white muscle? |
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Definition
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|
Term
what is the fate of glycogen in red muscle |
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Definition
pyruvate, acetyl coA, and ultimately CO2 |
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|
Term
where is teh enzyme glucose 6 phosphatase located? |
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Definition
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|
Term
how is glucose released into the blood? |
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Definition
a bidirectional facilitated glucose transporter (glut 1) at the plasma membrane |
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|
Term
what is the name of the activated glucose from which glycogen can be synthesized? |
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Definition
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|
Term
what makes UDP-glucose energetically favorable? |
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Definition
hydrolysis of inorganic pyrophosphate by pyrophosphatase |
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|
Term
what is the immediate donor of glucose residues to be added at the non reducing end of glycogen |
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Definition
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|
Term
what places glucose at the non reducing end of glycogen |
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Definition
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|
Term
this provides a primer for glycogen synthesis by glycogen synthase |
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Definition
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|
Term
what effect does phosphorylation have on glycogen phosphorylase and glycogen synthase? |
|
Definition
activates glycogen phosphorylase
inhibits glycogen synthase |
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|
Term
what form is glycogen synthase in when dephosphorylation occurs |
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Definition
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|
Term
when are liver glycogen levels high? |
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Definition
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|
Term
how long does stored glycogen last? |
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Definition
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|
Term
what does muscle do with free glucose? |
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Definition
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|
Term
is muscle glycogen important for maintenance of blood glucose in the post-absorbtive state |
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Definition
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|
Term
is muscle glycogen important for maintenance of blood glucose in the post-absorbtive state |
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Definition
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|
Term
what is the relationship between phoshphorylase a, glycogen synthase, and phosphoprotein phosphatase |
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Definition
phoshporylase a inhibits dephosphorylation of glycogen synthase b by phosphoprotein phosphatase |
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|
Term
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Definition
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|
Term
what is the relationship b/t phosphorylase and phosphoprotein phosphatase |
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Definition
phosphoprotein converts the phosphorylase to the inactive b form |
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|
Term
what is the relationship between phosphoprotein phosphatase and glycogen synthase |
|
Definition
the active form of phosphoprotein phosphatase activates glycogen synthase |
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|
Term
what is the relationship between phosphoprotein phosphatase and glycogen synthase |
|
Definition
the active form of phosphoprotein phosphatase activates glycogen synthase |
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|
Term
what is the relationship between glucagon and epinephrine in reference to glycogenolysis? |
|
Definition
It stimulates it by increasing intracellular cAMP |
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|
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 |
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|
Term
when epinephrine binds in the liver, what is the effect? |
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Definition
stimulation of glycogenolysis by generation of inositol 1,4,5 triphosphate and diaglycerol |
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|
Term
how does increased cytosolic Ca effect signaling? |
|
Definition
this activates phosphorylase kinase which activates glycogen phosphorylase with glycogenolysis. |
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|
Term
how does epinephrine affect signaling in the heart and skeletal muscle? |
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Definition
cAMP-->PKA-->phos. of phosphorylase kinase-->which phos. and activates glycogen phosphorylase. |
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|
Term
what activates muscle contraction? |
|
Definition
intracellular Ca is increased and that activates phosphorylase kinase and glycogen phosphorylase |
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|
Term
what kind of transporter does hepatocytes have? |
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Definition
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|
Term
what does glut2 transporter allow? |
|
Definition
uptake of glucose to be dependent on the glucose concentration in the blood. |
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|
Term
how does insulin stimulate glycogen synthesis |
|
Definition
activating phosphoprotein phosphotase which in turn dephosphorylates glycogen synthase. |
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|
Term
what does insulin induce in the muscle on its membrane?> |
|
Definition
glut4 transporter-->glycogenesis |
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|
Term
what are the three types of liver glycogen storage diseases? |
|
Definition
von gierke's disease, limit dextrinosis, amylopectinosis |
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|
Term
what is the enzyme deficiency in Von Gierke's disease? |
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Definition
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|
Term
what is the enzyme deficiency in limit dextrinosis? |
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Definition
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|
Term
what is the enzyme deficiency in amylopectinosis? |
|
Definition
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|
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. |
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|
Term
what are the name of the glycogen storage diseases that affect the muscle? |
|
Definition
McArdle's syndrome and Pompe's disease |
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|
Term
what is the enzyme deficiency in McArdle's syndrome |
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Definition
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|
Term
what are some clinical manifestations of McArdle's syndrome |
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Definition
exercise intolerance, intense myoglobinuria |
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