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

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(...

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Vydáno v:Cell Rep
Hlavní autoři: Sullivan, Mitchell A., Nitschke, Silvia, Skwara, Evan P., Wang, Peixiang, Zhao, Xiaochu, Pan, Xiao S., Chown, Erin E., Wang, Travis, Perri, Ami M., Lee, Jennifer P.Y., Vilaplana, Francisco, Minassian, Berge A., Nitschke, Felix
Médium: Artigo
Jazyk:Inglês
Vydáno: 2019
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Article
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC6530600/
https://ncbi.nlm.nih.gov/pubmed/31042462
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.celrep.2019.04.017
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