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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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Опубликовано в: :Sci Rep
Главные авторы: 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.
Формат: Artigo
Язык:Inglês
Опубликовано: Nature Publishing Group 2017
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Online-ссылка:https://ncbi.nlm.nih.gov/pmc/articles/PMC5357851/
https://ncbi.nlm.nih.gov/pubmed/28317891
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/srep44408
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