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Glycogen storage disease type IV is a very rare hereditary metabolic disorder.
It is also known as:-
The eponym “Andersen’s disease” is sometimes used, for Dorothy Hansine Andersen.[1][2][3]
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 amylose) 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.
See main article: Glycogen branching enzyme deficiency
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