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Glycogen stroage disease mnemonic

7K views 7 replies 5 participants last post by  struggle 
#1 ·
Hi, does anyone have a good way to memorize all six glycogen storage disease?
 
#2 · (Edited)
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
 
#6 ·
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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#8 ·
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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