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), the separation of soluble and insoluble muscle glycogen is described, enabling separate analysis of each fraction.
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