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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Detalles Bibliográficos
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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2019
Materias:
Original Articles
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

Internet

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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