Article
Antioxidants successfully reduce ROS production in propionic acidemia fibroblasts.
Biochemical and biophysical research communications - 26 Sept 2014
Gallego-Villar Lorena, Pérez Belén, Ugarte Magdalena, Desviat Lourdes R, Richard Eva
Abstract excerpt
Propionic acidemia (PA), caused by a deficiency of the mitochondrial biotin dependent enzyme propionyl-CoA carboxylase (PCC) is one of the most frequent organic acidurias in humans. Most PA patients present in the neonatal period with metabolic acidosis and hyperammonemia, developing different neurological symptoms, movement disorders and cardiac complications. There is strong evidence indicating that oxidative...
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