Normalization of hepatic ChREBP activity does not protect against liver disease progression in a mouse model for Glycogen Storage Disease type Ia

BACKGROUND: Glycogen storage disease type 1a (GSD Ia) is an inborn error of metabolism caused by a defect in glucose-6-phosphatase (G6PC1) activity, which induces severe hepatomegaly and increases the risk for liver cancer. Hepatic GSD Ia is characterized by constitutive activation of Carbohydrate R...

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Detalles Bibliográficos
Autores principales: Rutten, Martijn G. S., Lei, Yu, Hoogerland, Joanne H., Bloks, Vincent W., Yang, Hong, Bos, Trijnie, Krishnamurthy, Kishore A., Bleeker, Aycha, Koster, Mirjam H., Thomas, Rachel E., Wolters, Justina C., van den Bos, Hilda, Mithieux, Gilles, Rajas, Fabienne, Mardinoglu, Adil, Spierings, Diana C. J., de Bruin, Alain, van de Sluis, Bart, Oosterveer, Maaike H.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10122297/
https://www.ncbi.nlm.nih.gov/pubmed/37085901
http://dx.doi.org/10.1186/s40170-023-00305-3

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