21 - Glycogen metabolism – structure, synthesis and degradation. Regulation – mechanisms. Glycogen storage diseases.

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7 Terms

1
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sections

  • glycogen metabolism

  • structure

  • degradation

  • regulatory enzyme

  • deficiency

  • oral box

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  • glycogen metabolism

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  • structure

  • polymer of glucose

  • sphere shape

  • chains are branched (inner) and unbranched (outer)

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  • degradation (glycogenolysis)

  1. glycogen phosphorylase cleaves 1-4 glycosidic bonds

  2. glucose-1-phosphate released from glycogen

  3. remodelling of glycogen for further degradation

  4. conversion of glucose-1-p to glucose-6-p for further metabolism

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  • regulatory enzyme

  • glycogen synthase

  • glycogen phosphorylase

  • phosphoglucomutase

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  • deficiency

4 types

  1. Von Glerkes - hypoglycaemia

  2. Andersons - liver dysfunction

  3. McArdle’s - muscle cramps when exercising

  4. Muscle deficiency of phosphofructokinase - inability to exercise

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<ul><li><p>oral box</p></li></ul><p>deficiencies are same for both ( the last box, missing in pic)</p>

  • oral box

deficiencies are same for both ( the last box, missing in pic)