Glycogen Storage Diseases and Glycogenesis

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Which enzyme catalyzes the conversion of glucose to Glucose-6-Phosphate in muscle?

Hexokinase

What is the main function of UDPGlc pyrophosphorylase?

Catalyzing glycogen synthesis

Which organ mainly utilizes glucokinase for the conversion of glucose to Glucose-6-Phosphate?

Liver

What is the immediate precursor of UDPGlc in glycogenesis?

Glucose-1-Phosphate

Which process involves the transfer of a phosphate group from the sixth carbon to the first carbon of the glucose molecule?

Isomerization to Glucose-1-Phosphate

What is the final product formed by the catalytic action of UDPGlc pyrophosphorylase?

Uridine diphosphate glucose (UDPGlc)

What is the main enzyme that catalyzes the rate-limiting step in glycogenolysis?

Glycogen phosphorylase

What is the purpose of the debranching enzyme in glycogenolysis?

To transfer a trisaccharide unit from one branch to another, exposing the 1,6 branch point

What is the role of phosphoglucomutase in glycogenolysis?

It catalyzes the conversion of glucose 1-phosphate to glucose-6-phosphate, and the reverse reaction is also possible

Which of the following statements about the differences between glycogenolysis and glycogenesis is correct?

Glycogenolysis and glycogenesis are separate pathways that use different sets of enzymes

What is the role of glucose-6-phosphatase in glycogenolysis in the liver?

It catalyzes the hydrolysis of glucose-6-phosphate to yield free glucose, which is then exported to increase blood glucose concentration

What is the final outcome of the combined action of phosphorylase and the other enzymes involved in glycogenolysis?

The complete breakdown of glycogen to yield free glucose

What is the role of 5' AMP in muscle phosphorylase?

It activates the inactive form (phosphorylase b) of the enzyme

What is the primary signal that leads to increased cAMP formation in liver cells?

Glucagon secretion

Which of the following is an allosteric inhibitor of active phosphorylase A in both liver and muscle tissues?

ATP

What is the role of cAMP in the activation of glycogen phosphorylase?

It activates phosphorylase kinase a, which then activates phosphorylase b

What is the primary signal that leads to increased cAMP formation in muscle cells?

Norepinephrine secretion

Which of the following is an allosteric inhibitor of active phosphorylase A in liver, but not in muscle?

Free glucose

What enzyme catalyzes the formation of a glycoside bond between C-1 of the glucose of UDPGlc and C-4 of a terminal glucose residue of glycogen?

Glycogen synthase

At which end of the glycogen molecule does the addition of a glucose residue to a preexisting glycogen chain occur?

Nonreducing, outer end

What is the minimum length of a growing glycogen chain before the branching enzyme can transfer a part of the 1 4-chain to a neighboring chain to form a 1 6 linkage?

11 glucose residues

What is the role of insulin in the regulation of glycogen metabolism?

Insulin decreases the level of cAMP only after it has been raised by glucagon or epinephrine

Which hormone is active in heart muscle but not in skeletal muscle?

Glucagon

What is the relationship between the enzymes glucan transferase and debranching enzyme?

Glucan transferase and debranching enzyme appear to be two separate activities of the same enzyme

How is phosphorylase A inactivated?

By protein phosphatase-1

What inhibits protein phosphatase-1?

Inhibitor-1 phosphorylated by cAMP-dependent protein kinase

How does insulin reinforce the inactivation of phosphorylase b?

By increasing glucose uptake

What happens to glycogen synthase a to make it active?

It gets dephosphorylated

How many different protein kinases act on glycogen synthase?

Six

What type of hormonal action allows the inhibition of glycogen synthesis along with the activation of glycogenolysis?

cAMP-mediated hormonal action

Learn about glycogen storage diseases, conditions caused by deficient mobilization or abnormal deposition of glycogen, leading to liver damage and muscle weakness. Explore the process of glycogenesis, involving UDP-Glucose and the conversion of glucose to Glucose-6-Phosphate (G6P) in muscle and liver.

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