Altered Redox Homeostasis in Branched-Chain Amino Acid Disorders, Organic Acidurias, and Homocystinuria

@inproceedings{Richard2018AlteredRH,
  title={Altered Redox Homeostasis in Branched-Chain Amino Acid Disorders, Organic Acidurias, and Homocystinuria},
  author={E. Chaisson Richard and Lorena Gallego-Villar and Ana Rivera-Barahona and A. Mi{\~n}ano Oyarzabal and Bel{\'e}n P{\'e}rez and Pilar Rodr{\'i}guez-Pombo and Lourdes R Desviat},
  booktitle={Oxidative medicine and cellular longevity},
  year={2018}
}
Inborn errors of metabolism (IEMs) are a group of monogenic disorders characterized by dysregulation of the metabolic networks that underlie development and homeostasis. Emerging evidence points to oxidative stress and mitochondrial dysfunction as major contributors to the multiorgan alterations observed in several IEMs. The accumulation of toxic metabolites in organic acidurias, respiratory chain, and fatty acid oxidation disorders inhibits mitochondrial enzymes and processes resulting in… CONTINUE READING
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