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Article

Skeletal Muscle Glycogen Chain Length Correlates with Insolubility in Mouse Models of Polyglucosan-Associated Neurodegenerative Diseases

1 Apr 2019

Abstract excerpt

Lafora disease (LD) and adult polyglucosan body disease (APBD) are glycogen storage diseases characterized by a pathogenic buildup of insoluble glycogen. Mechanisms causing glycogen insolubility are poorly understood. Here, in two mouse models of LD ( Epm2a −/− and Epm2b −/− ) and one of APBD ( Gbe1 ys/ys ), the separation of soluble and insoluble muscle glycogen is described, enabling separate analysis of each...

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Identifiers

PMID: 31042462
DOI: 10.1016/j.celrep.2019.04.017

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