The Effect of Methylmalonic Acid Treatment on Human Neuronal Cell Coenzyme Q<sub>10</sub> Status and Mitochondrial Function

The Effect of Methylmalonic Acid Treatment on Human Neuronal Cell Coenzyme Q<sub>10</sub> Status and Mitochondrial Function

Methylmalonic acidemia is an inborn metabolic disease of propionate catabolism, biochemically characterized by accumulation of methylmalonic acid (MMA) to millimolar concentrations in tissues and body fluids. However, MMA’s role in the pathophysiology of the disorder and its status as a “toxic inter...

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Bibliographic Details
Main Authors: Emma C. Proctor, Nadia Turton, Elle Jo Boan, Emily Bennett, Suzannah Philips, Robert A. Heaton, Iain P. Hargreaves
Format: Article
Language:English
Published: MDPI AG 2020-11-01
Series:International Journal of Molecular Sciences
Subjects:
coenzyme Q<sub>10</sub>
mitochondrial respiratory chain
methylmalonic acidemia
mitochondrial dysfunction
mitochondrial membrane potential
Online Access:https://www.mdpi.com/1422-0067/21/23/9137

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https://www.mdpi.com/1422-0067/21/23/9137

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