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Liver Glycogen Phosphorylase Deficiency Leads to Profibrogenic Phenotype in a Murine Model of Glycogen Storage Disease Type VI

Mutations in the liver glycogen phosphorylase (Pygl) gene are associated with the diagnosis of glycogen storage disease type VI (GSD‐VI). To understand the pathogenesis of GSD‐VI, we generated a mouse model with Pygl deficiency (Pygl (−/−)). Pygl (−/−) mice exhibit hepatomegaly, excessive hepatic gl...

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Dettagli Bibliografici
Pubblicato in:Hepatol Commun
Autori principali: Wilson, Lane H., Cho, Jun‐Ho, Estrella, Ana, Smyth, Joan A., Wu, Rong, Chengsupanimit, Tayoot, Brown, Laurie M., Weinstein, David A., Lee, Young Mok
Natura: Artigo
Lingua:Inglês
Pubblicazione: John Wiley and Sons Inc. 2019
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC6824077/
https://ncbi.nlm.nih.gov/pubmed/31701076
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/hep4.1426
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