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Nicotinamide riboside rescues dysregulated glycolysis and fatty acid β-oxidation in a human hepatic cell model of citrin deficiency

Citrin deficiency (CD) is an inborn error of metabolism caused by loss-of-function of the mitochondrial aspartate/glutamate transporter, CITRIN, which is involved in both the urea cycle and malate–aspartate shuttle. Patients with CD develop hepatosteatosis and hyperammonemia but there is no effectiv...

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Detalles Bibliográficos
Autores principales: Yau, Winifred W, Chen, Gao Bin, Zhou, Jin, Francisco, Joel Celio, Thimmukonda, Nivetha Kanakaram, Li, Shang, Singh, Brijesh Kumar, Yen, Paul Michael
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10196666/
https://www.ncbi.nlm.nih.gov/pubmed/36881658
http://dx.doi.org/10.1093/hmg/ddad018