Glycogenesis and storage diseases

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Questions and Answers

What is the primary function of muscle glycogen?

  • To store glucose in animals
  • To regulate glycogen metabolism
  • To maintain blood glucose levels between meals
  • To serve as a fuel reserve for the supply of ATP during muscle contraction (correct)

What type of linkages hold glucose molecules together in glycogen?

  • α-1,4 and β-1,6
  • β-1,4 and β-1,6
  • β-1,4 and α-1,6
  • α-1,4 and α-1,6 (correct)

What happens to glycogen stores during fasting?

  • They remain constant
  • They increase
  • They are depleted (correct)
  • They are converted to protein

What is the percentage of glycogen stored in the liver?

<p>6-8% (A)</p> Signup and view all the answers

What is the overall function of glycogen in animals?

<p>To store glucose in animals (A)</p> Signup and view all the answers

What is the primary purpose of glycogenesis in liver and muscle?

<p>To store excess glucose as glycogen (D)</p> Signup and view all the answers

Which enzyme is responsible for transferring the first glucose molecule to glycogenin?

<p>Glycogen initiator synthase (D)</p> Signup and view all the answers

What is the carrier of glucose molecules during glycogenesis?

<p>UDPG (A)</p> Signup and view all the answers

What is the name of the protein that can accept glucose from UDPG in the absence of a glycogen primer?

<p>Glycogenin (B)</p> Signup and view all the answers

Which enzyme is responsible for synthesizing glycogen from glucose in liver and muscle?

<p>Glycogen synthase (D)</p> Signup and view all the answers

What is the site of attachment of glucose from UDPG to glycogenin?

<p>OH group of tyrosine residue of glycogenin (B)</p> Signup and view all the answers

What is the primary function of the branching enzyme in glycogen synthesis?

<p>To transfer glucose residues from one chain to a neighboring chain (C)</p> Signup and view all the answers

What is the end product of liver glycogenolysis?

<p>Glucose (A)</p> Signup and view all the answers

What is the role of phosphorylase in glycogenolysis?

<p>To phosphorolytically split α-1,4 glucoside bonds (B)</p> Signup and view all the answers

What is the name of the enzyme that splits the α-1,6 linkages in glycogenolysis?

<p>Amylo 1,6 glucosidase (D)</p> Signup and view all the answers

In which tissues does glycogenolysis occur?

<p>In both liver and muscle (D)</p> Signup and view all the answers

What is the role of phosphoglucomutase in glycogenolysis?

<p>To convert glucose-1-phosphate to glucose-6-phosphate (B)</p> Signup and view all the answers

What is the role of high cAMP levels in the regulation of glycogenesis and glycogenolysis?

<p>It activates phosphorylase and inhibits glycogen synthase (B)</p> Signup and view all the answers

What is the effect of gluc-6-P on phosphorylase activity?

<p>It inhibits phosphorylase (A)</p> Signup and view all the answers

What is the role of protein phosphatase in the regulation of glycogen metabolism?

<p>It converts glycogen synthase a to glycogen synthase b (A)</p> Signup and view all the answers

What is the effect of adrenaline on glycogen metabolism?

<p>It activates phosphorylase (D)</p> Signup and view all the answers

What is the role of insulin in glycogen metabolism?

<p>It activates glycogen synthase (D)</p> Signup and view all the answers

What is the characteristic of glycogen storage diseases?

<p>They are genetic diseases that result in the deposition of abnormal or increased quantity of glycogen (A)</p> Signup and view all the answers

What is the primary consequence of a block in gluconeogenesis in Von Gierke's disease?

<p>Mobilization of fat to meet energy requirements (D)</p> Signup and view all the answers

Which enzyme is responsible for the accumulation of glycogen in lysosomes in all tissues, including muscles?

<p>Lysosomal α-1, 4-glucosidase (C)</p> Signup and view all the answers

What is the consequence of accumulated glucose-6-phosphate in Von Gierke's disease?

<p>Diversion to HMP pathway, leading to increased synthesis of ribose and nucleotides (B)</p> Signup and view all the answers

What is the primary reason for lactic acidemia in Von Gierke's disease?

<p>Glucose is not synthesized from lactate produced in muscle and liver (B)</p> Signup and view all the answers

Which enzyme is responsible for the accumulation of polysaccharide with few branch points?

<p>Debranching enzyme (C)</p> Signup and view all the answers

What is the consequence of massive liver enlargement in Von Gierke's disease?

<p>Cirrhosis of the liver (B)</p> Signup and view all the answers

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