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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...
Autores principales: | Wilson, Lane H., Cho, Jun‐Ho, Estrella, Ana, Smyth, Joan A., Wu, Rong, Chengsupanimit, Tayoot, Brown, Laurie M., Weinstein, David A., Lee, Young Mok |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6824077/ https://www.ncbi.nlm.nih.gov/pubmed/31701076 http://dx.doi.org/10.1002/hep4.1426 |
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