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Old 03-10-2011
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Help Glycogen stroage disease mnemonic

Hi, does anyone have a good way to memorize all six glycogen storage disease?
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Old 03-10-2011
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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

Last edited by mary; 03-10-2011 at 09:44 AM. Reason: Correction and modification
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Old 03-10-2011
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Thanks Mary,

I copied your post to our Step 1 Mnemonics Forum here's the link
Gylcogen Storage Diseases Mnemonic
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Old 03-10-2011
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Thank you, Mary. That is very helpful.

In addition, I would put Anderson (IV) in with Metabolism .
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Old 03-10-2011
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Quote:
Originally Posted by usluipek View Post
Thank you, Mary. That is very helpful.

In addition, I would put Anderson (IV) in with Metabolism .
Thank you for the note
I added Anderson, see above
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Old 03-11-2011
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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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Old 03-11-2011
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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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