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Hepatic mitochondrial dysfunction is a feature of Glycogen Storage Disease Type Ia (GSDIa)

Glycogen storage disease type Ia (GSDIa, von Gierke disease) is the most common glycogen storage disorder. It is caused by the deficiency of glucose-6-phosphatase, an enzyme which catalyses the final step of gluconeogenesis and glycogenolysis. Clinically, GSDIa is characterized by fasting hypoglycae...

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Detalles Bibliográficos
Autores principales:	Farah, Benjamin L., Sinha, Rohit A., Wu, Yajun, Singh, Brijesh K., Lim, Andrea, Hirayama, Masahiro, Landau, Dustin J., Bay, Boon Huat, Koeberl, Dwight D., Yen, Paul M.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group 2017
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5357851/ https://www.ncbi.nlm.nih.gov/pubmed/28317891 http://dx.doi.org/10.1038/srep44408

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