Glycogen: Structure, Location & Function

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Questions and Answers

Why is glycogen a more advantageous form of carbohydrate storage compared to free-floating glucose?

  • Storing glucose as glycogen prevents excessive osmotic pressure within the cell. (correct)
  • Glycogen directly enters the bloodstream, bypassing cellular metabolism.
  • Glycogen is more readily available for immediate energy use.
  • Free-floating glucose is more stable and less prone to degradation.

Which of the following is NOT a primary location for glycogen synthesis and storage in the body?

  • Kidney
  • Brain (correct)
  • Skeletal Muscle
  • Liver

What is the primary function of muscle glycogen?

  • Synthesizing hormones for systemic distribution.
  • Serving as an energy reserve specifically for muscle contraction. (correct)
  • Detoxifying harmful substances in the bloodstream.
  • Maintaining blood glucose levels during fasting.

In glycogen synthesis, what is the role of glycogenin?

<p>It acts as the core protein on which glucose molecules are initially added. (C)</p>
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Why is glycogen branching important?

<p>It creates more non-reducing ends for rapid glucose mobilization. (C)</p>
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What type of glycosidic bond does glycogen phosphorylase act on?

<p>α-1,4-glycosidic bonds (B)</p>
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Which of the following statements accurately describes the function of the debranching enzyme in glycogenolysis?

<p>It transfers a limit branch of oligosaccharides and hydrolyzes the α-1,6 glycosidic bond. (D)</p>
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In which scenario would glycogen breakdown in the liver be most critical?

<p>During prolonged fasting. (C)</p>
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What is the immediate product of glycogen phosphorylase activity?

<p>Glucose-1-phosphate (A)</p>
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What is the role of phosphoglucomutase?

<p>It converts glucose-1-phosphate to glucose-6-phosphate. (A)</p>
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If an individual has a genetic defect resulting in a non-functional branching enzyme, what would be the most likely consequence?

<p>Formation of glycogen with fewer branch points and decreased solubility. (C)</p>
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During exercise, epinephrine stimulates glycogen breakdown in muscle. What is the primary purpose of this process?

<p>To provide energy for muscle contraction. (D)</p>
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What is a key characteristic of McArdle disease?

<p>It is caused by a deficiency in muscle glycogen phosphorylase, leading to exercise-induced muscle pain. (B)</p>
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Which of the following best describes the role of UDP-glucose pyrophosphorylase in glycogen synthesis?

<p>It converts glucose-1-phosphate and UTP into UDP-glucose and pyrophosphate. (D)</p>
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How does the function of glycogen synthase differ from that of branching enzyme?

<p>Glycogen synthase adds glucose to the linear chain, while branching enzyme creates α-1,6 linkages. (C)</p>
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If a patient is diagnosed with Von Gierke's disease (Type Ia), which enzyme is most likely deficient?

<p>Glucose-6-phosphatase (D)</p>
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What is the metabolic consequence of a deficiency in the debranching enzyme?

<p>The inability to effectively degrade glycogen at branch points. (A)</p>
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In white (fast-twitch) muscle fibers, glucose is primarily converted to lactate. Why?

<p>The rate of glycolysis exceeds the capacity of the mitochondria to oxidize pyruvate. (D)</p>
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How does the allosteric regulation of glycogen phosphorylase contribute to energy homeostasis?

<p>It allows glycogen breakdown to be responsive to the energy needs of the cell. (B)</p>
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Which of the following enzymes is unique to the liver and allows it to release free glucose into the bloodstream?

<p>Glucose-6-phosphatase (B)</p>
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Flashcards

What is glycogen?

Glycogen is a branched polymer of glucose and is the storage form of glucose in animals and humans.

Where does glycogen synthesis occur?

Glycogen synthesis and degradation occur primarily in the liver and skeletal muscle.

Muscle glycogen's purpose?

In muscle cells, glycogen is stored as an energy reserve for muscle contraction.

Liver glycogen function?

The liver stores glycogen as a source of glucose that can be mobilized during hypoglycemia.

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How is glycogen stored?

Glycogen is stored in the cytoplasm as either single granules (skeletal muscle) or as clusters of granules (liver). Each granule has a central protein core with polyglucose chains radiating outward to form a spherical structure.

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Key Enzyme in Glycogen Synthesis?

Glycogen synthase is the rate-limiting enzyme in glycogen synthesis.

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Glycogen phosphorylase action?

Glycogen phosphorylase breaks α-1,4-glycosidic bonds, releasing glucose 1-phosphate from the periphery of the granule.

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Glycogen phosphorylase limitations?

Glycogen phosphorylase cannot break α-1,6 bonds and therefore stops when it nears the outermost branch points.

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Debranching enzyme function?

Debranching enzyme deconstructs the branches in glycogen that have been exposed by glycogen phosphorylase. It breaks α-1,4 bond adjacent to the branch point and hydrolyzes the α-1,6 bond.

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McArdle disease

McArdle disease is a genetic disorder that mainly affects skeletal muscles due to the deficiency or absence of the enzyme myophosphorylase. Symptoms include painful muscle cramps, weakness, and fatigue manifest during periods of physical activity.

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Study Notes

  • Glycogen is a branched polymer of glucose and acts as a storage form of glucose in the body
  • Glycogen synthesis and degradation primarily occur in the liver and skeletal muscle, but can occur in other tissues, including cardiac muscle and the kidney, which store smaller quantities
  • Storing carbohydrates as glycogen has many advantages in comparison to storing free-floating glucose

Glycogen Location and Structure

  • Glycogen is stored in the cytoplasm as single granules in skeletal muscle or as clusters of granules in the liver
  • Each granule is composed of a central protein core surrounded by polyglucose chains radiating outward in a spherical structure
  • Liver glycogen serves as a source of glucose that is mobilized during hypoglycemia, aiding in maintenance of blood sugar levels
  • Muscle glycogen acts as an energy reserve for muscle contraction
  • In white (fast-twitch) muscle fibers, glucose turns primarily to lactate
  • In red (slow-twitch) muscle fibers, glucose is completely oxidized

Glycogen Synthesis

  • Glycogen synthesis (glycogenesis) and glycogen breakdown (glycogenolysis) are the 2 pathways involved in glycogen metabolism
  • Glycogen synthesis starts with the core protein glycogenin, which helps begin the formation of glycogen granules
  • Glucose in the form of glucose 6-phosphate gets turned into glucose 1-phosphate and activated to UDP-glucose
  • UDP-glucose is added to glycogen chains by glycogen synthase
  • Glycogen synthase is the rate-limiting enzyme in glycogen synthesis

Glycogenolysis

  • Glycogen phosphorylase is the rate-limiting enzyme involved in glycogenolysis (glycogen breakdown)
  • Glycogen phosphorylase is a phosphorlase, which breaks bonds using Pi rather than H20
  • Glycogen phosphorylase breaks α-1,4 glycosidic bonds, releasing glucose 1-phosphate from the periphery of the granule
  • The glucose 1-phosphate that is formed gets converted to glucose 6-phosphate by the mutase that is utilized in glycogen synthesis

Debranching Enzyme

  • Debranching enzymes deconstruct glycogen branches that were already exposed by glycogen phosphorylase through 2 mechanisms:
  • Breaking α-1,4 bonds adjacent to branch points and moving small oligoglucose chains that have been released to exposed ends of other chains, forming new α-1,4 bonds
  • Hydrolyzing α-1,6 bonds, releasing single residues at the branch point as free glucose

Genetic Deficiencies

  • Many genetic deficiencies of enzymes in glycogen metabolism have been classified as glycogen storage diseases
  • Glycogen storage diseases are characterized by the accumulation of glycogen in one or more tissues
  • McArdle disease mainly affects skeletal muscles and happens because of a deficiency or absence of myophosphorylase
  • Symptoms include painful muscle cramps, weakness, and fatigue that occur during physical activity

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