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

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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Bibliographic Details
Main Authors: Igor Radzikh, Erica Fatica, Jillian Kodger, Rohan Shah, Ryan Pearce, Yana I. Sandlers
Format: Article
Language:English
Published: MDPI AG 2021-08-01
Series:Metabolites
Subjects:
VLCAD deficiency
dodecandioic acid
acylcarnitines
Krebs cycle
Online Access:https://www.mdpi.com/2218-1989/11/8/538

Internet

https://www.mdpi.com/2218-1989/11/8/538

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