Glycogen storage disease type IV

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Glycogen storage disease type IV
Glycogen
ICD-10	E74.0
ICD-9	271.0
OMIM	232500
DiseasesDB	5303
MeSH	C16.320.565.202.449.540

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 ; Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]

Synonyms and keywords: Glycogenosis type IV , Andersen's disease , glycogen branching enzyme deficiency (GBED , polyglucosan body disease, amylopectinosis

Glycogen storage disease type IV is a very rare hereditary metabolic disorder.

Human pathology

It is a result of the absence of the glycogen branching enzyme amylo-1,4-1,6 transglucosidase, which is critical in the production of glycogen. This leads to very long unbranched glucose chains being stored in glycogen. The long unbranched molecules (known as amylopectin) have a low solubility which leads to glycogen precipitation in the liver. These deposits subsequently build up in the body tissue, especially the heart and liver. The end result is liver failure and eventual death occurring in the first year of life.

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