Glycogen Questions Flashcards

1
Q

What is the general structure of glycogen?

A

It consists of glucose connected by a-1,4 glycosidic linkages with branches at 1-1,6 glycosidic bonds, branches distributing at even intervals resulting in spherical structure.

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2
Q

What is the general mechanism of glycogen build-up?

A

When glucose enters the cell, it is phosphorylated into G6P, then isomerised into G1P by phosphoglucomutase-1 wheren then UDP-glucose is catalysed from this by UDP glucose pyro-phosphorylase, serving as an intermediate glucose donor for glycogen construction.

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3
Q

What initiates glycogen, and what initially elongates it?

A

Glycogenin, elongated by glycogen synthase, then branched by GDE introducing 1-1,6 glycosidic bonds at regular intervals.

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4
Q

How is glycogen breakdown cytosilically achieved?

A

Co-ordination of glycogen phosphorylase which release glucose-1 phosphate by removal of the a-1,4 glycosidic linkage, and GBE.

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5
Q

What happens to G1P derived from the cytosolic pathway proceeding glycogen degradation?

A

Isomerised into G6P, which can bed dephosphorylated to free glucose by the enzyme glucose 6 phosphotase, where glucose transporters secrete it.

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6
Q

How is glucose uptake facilitated in the brain?

A

GLUT1 across the endothelium of the BBB, accounting for majority of glucose taken up by the body.

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7
Q

How is glucose uptake facilitated in the skeletal muscle?

A

GLUT 1 on PM facilitating transport into the muscle fibre and GLUT4, found within intracellular storage vesicles which in muscle contraction/insulin stimulation, translocate into the PM.

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8
Q

How is glucose uptake facilitated in the Liver/Pancreas?

A

GLUT2 absorbs glucose from blood to tissue.

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9
Q

What does Franconi-Bickel disease derive from?

A

GLUT2 gene mutations with impaired utilization of glucose/galactose, leading to both hyperglycemia an hypergalactosemia.

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10
Q

What is the first enzyme involved in conversion of free glucose to G6P?

A

Hexokinase.

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11
Q

What do GKRP do in fasting?

A

They inhibit glucokinase action, limiting glucose utilization.

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12
Q

What do glucokinase mutations lea to?

A

Disordered glucose metabolism resulting in hypoglycemia

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13
Q

What does glucokinase deficiency lead to?

A

Hinderance of pancreation/hepatocyte glucose metabolism, leading to impaired insulin secretion from the pancrease, reducing glycogen synthesis in the liver

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14
Q

How is UDP-Glucose formed and when does it take place?

A

Formed from catalysis by UDP glucose pyrophosphatase, facilitating reversible conversion to UDP-glucose from reactants; UTP and G1P, in the presence of Mg2+., following G1P formation.

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15
Q

What is glycogenin?

A

A transferase catalysing transfer of glucose residues from the UDP-glucose to itself, forming the a-1,4 glycosidic linkages creating linear glucose polymers of about 10-20 glucose.

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16
Q

What is the importance of glycogenin?

A

Building foundation for synthesis of glycogen by glycogen synthase and the GBE.

17
Q

What does glycogen synthase deficiency in the muscle lead to?

A

Glycogen depletion in skeletal muscle and the heart, resultign in myopathy.

18
Q

How is glycogen synthase regulated?

A

By reversible phosphorylation, catalysed by various kinases that inactive it, and dephosphorylation, catalysed by phosphatase enzymes, activating it.

19
Q

What activates protein phosphatase-1?

A

Insulin

20
Q

What is the general structure of protein phosphatase-1?

A

A catalytic subunit bound to a regulatory subunit modulating catalytic action by targeting the protein to particular sub-cellular locations and specific substrates.

21
Q

How does G6P effect hepatic glcyogen synthase activity?

A

G6P is an allosteric activatior of glycogen synthase, also converting the enzyme into a better substrate for protein phosphatases, leading to further activation of glycogen synthase.

22
Q

What is the process of glycogen breakdown in the cytosol?

A

Glycogen phosphorylas results in G1P release from glycogen chain, where GDE completely degrades it.