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An evolutionary approach to optimizing glucose-6-phosphatase-α enzymatic activity for gene therapy of glycogen storage disease type Ia

Glycogen storage disease type-Ia (GSD-Ia), caused by a deficiency in glucose-6-phosphatase-α (G6Pase-α or G6PC), is characterized by impaired glucose homeostasis with a hallmark hypoglycemia, following a short fast. We have shown that G6pc-deficient (G6pc−/−) mice treated with recombinant adeno-asso...

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Bibliographische Detailangaben
Veröffentlicht in:J Inherit Metab Dis
Hauptverfasser: Zhang, Lisa, Cho, Jun-Ho, Arnaoutova, Irina, Mansfield, Brian C., Chou, Janice Y.
Format: Artigo
Sprache:Inglês
Veröffentlicht: 2019
Schlagworte:
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6483894/
https://ncbi.nlm.nih.gov/pubmed/30714174
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/jimd.12069
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