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Hepatic glucose-6-phosphatase-α deficiency leads to metabolic reprogramming in glycogen storage disease type Ia

Glycogen storage disease type Ia (GSD-Ia) is caused by a deficiency in glucose-6-phosphatase-α (G6Pase-α or G6PC), a key enzyme in endogenous glucose production. This autosomal recessive disorder is characterized by impaired glucose homeostasis and long-term complications of hepatocellular adenoma/c...

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Detalhes bibliográficos
Publicado no:Biochem Biophys Res Commun
Main Authors: Cho, Jun-Ho, Kim, Goo-Young, Mansfield, Brian C., Chou, Janice Y.
Formato: Artigo
Idioma:Inglês
Publicado em: 2018
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5905716/
https://ncbi.nlm.nih.gov/pubmed/29545180
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.bbrc.2018.03.083
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