Glycogen Catabolism Flashcards

1
Q

What is glycogen ?

A

A storage polysaccharide.
A very large polymer of glucose (alpha 1-4) and (alpha 1-6 linkage)
55,000 glucose residues - developing large alpha rosettes of glycogen granules

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2
Q

Who made major discoveries on glycogen metabolism?

A

The Coris

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3
Q

Where are large alpha rosettes found?

A

In the liver and in muscle cells.

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4
Q

What does glycogen do?

A
  • stored during meals ‘metered out’ during day.
  • mobilised storage form of glucose.
  • controlling blood glucose
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5
Q

What is the main catabolic enzyme in glycogen catabolism?

A

Glycogen phosphorylase - very highly regulated.

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6
Q

What is the role of glycogen phosphorylase enzyme?

A

It removes the terminal glucose residues from non-reducing end by phosphorolysis.
Adds a phosphoryl group.
Creates lots of glucose 1-phosphate.

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7
Q

Describe the debranching enzyme in the role of glycogen catabolism?

A

Glycogen phosphorylase acts until it meets the alpha 1-6 branch and stops working 4 residues before end.
Transferase activity - ‘debranching enzyme’ removes 3 glucose residues before branch to another tier.
Alpha 1-6 glucosidase activity of debranching enzyme cuts off glucose and continues on branch,

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8
Q

What is the final product of glycogen catabolism?

A

Lots of glucose 1 - phosphate which phosphoglucomutase converts to glucose 6-phosphate.

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9
Q

Describe the role of glucose 6-phosphate to becoming energy in the body/glycolysis? In liver?

A

In liver = glucose 6-phosphatase
Becomes glucose - blood
Transports to tissues = glycolysis.

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10
Q

Describe the role of glucose 6-phosphate to becoming energy in the body/glycolysis? In the muscle cells?

A

In muscle = glycolysis step 2 (anaerobic/aerobic)

= energy

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11
Q

What is the test for newborns for metabolic diseases?

A

The heel prick test, tests for rare usually inherited in autosomal recessive manner.

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12
Q

What are the 3 enzymes in which genetic mutations occur in glycogen storage?

A

glycogen phosphorylase
glycogen debranching enzyme
glycogen 6-phosphatase

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13
Q

What are some of the results and treatments for glycogen storage diseases?

A

big liver, myopathy, metabolic problems.

Treatment = low glucose/monitored carb diet.

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