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Metabolic Outcomes of Anaplerotic Dodecanedioic Acid Supplementation in Very Long Chain Acyl-CoA Dehydrogenase (VLCAD) Deficient Fibroblasts

Very long-chain acyl-CoA dehydrogenase deficiency (VLCADD, OMIM 609575) is associated with energy deficiency and mitochondrial dysfunction and may lead to rhabdomyolysis and cardiomyopathy. Under physiological conditions, there is a fine balance between the utilization of different carbon nutrients...

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Detalles Bibliográficos
Autores principales: Radzikh, Igor, Fatica, Erica, Kodger, Jillian, Shah, Rohan, Pearce, Ryan, Sandlers, Yana I.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8412092/
https://www.ncbi.nlm.nih.gov/pubmed/34436479
http://dx.doi.org/10.3390/metabo11080538