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Sirtuin signaling controls mitochondrial function in glycogen storage disease type Ia

Glycogen storage disease type Ia (GSD-Ia) deficient in glucose-6-phosphatase-α (G6Pase-α) is a metabolic disorder characterized by impaired glucose homeostasis and a long-term complication of hepatocellular adenoma/carcinoma (HCA/HCC). Mitochondrial dysfunction has been implicated in GSD-Ia but the...

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Bibliographic Details
Published in:J Inherit Metab Dis
Main Authors: Cho, Jun-Ho, Kim, Goo-Young, Mansfield, Brian C., Chou, Janice Y.
Format: Artigo
Language:Inglês
Published: 2018
Subjects:
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC6541525/
https://ncbi.nlm.nih.gov/pubmed/29740774
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s10545-018-0192-1
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