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MNEMONIC for Glycogen Storage Diseases:
Very Poor Casing And Metabolism of Hers

Note: casing refers to storage :)

  • I- Von Gierke's (Glu6P deficiency)
  • II- Pompe's (lysosmal 1,4 glucosidase)
  • III- Cori (1,6 glucosidase)
  • IV- Anderson (glycogen branching enzyme)
  • V- McArdles (Muscle GlycogenPhosphoylase )
  • VI- Hers disease (Hepatic Glycogen Phosphorylase)
Also this might help;

POMPe's trashes the PUMP (cardiomegaly)
HERS Disease- its HEpatic glycogen phosphorylase deficiency
McArdles- its Muscle Glycogen phosphorylase deficiency
 

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Very Poor Carbohydrate Metabolism (V,P,C,M) of Hers (H)
1-Von gierkes (Glu6P deficincy)
2-Pompes (lysosmal 1,4 glucosidase)
3-Cori (1,6glucosidase)
4- Mc Ardles (Muscle GlycogenPhosphoylase )
5-Hers disease (Hepatic Glycogen Phosphorylase)
Type 1 glycogen storage disease
Type 1 = one (Von), ie Von Giereke's disease
Glycogen storage: Anderson's (IV) vs. Cori's (III) enzyme defect
ABCD:
Anderson's=Branching enzyme.
Cori's=Debranching enzyme.
· Otherwise, can't really distinguish clinically.
 
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Reactions: Dr.Binish

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Discussion Starter · #7 ·
Hi, axax,

I do not quite understand why deficieincy in branching enzyme can cause glucogen storage disease. It seems all other glucogen storage diseases are caused by enzymes that are needed for glucogenolysis. So why branching enzyme?
 

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its because branching enzyme i.e.alpha 1;4:1;6 transferase..is required to break 1;4 bond and move it to 1;6 bond...branches have terminal glucose attached to it which can be released rapidly on demand..
also in linear glycogen there is just one terminal glucose while branched glycogen has 8-10% of R-terminal glucose..
hope this helps.
 
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