DNA damage induced by alloisoleucine and other metabolites in maple syrup urine disease and protective effect of l-carnitine.

  title={DNA damage induced by alloisoleucine and other metabolites in maple syrup urine disease and protective effect of l-carnitine.},
  author={Tatiane Cristina Hauschild and Gilian Guerreiro and Caroline Paula Mescka and Daniella de Moura Coelho and Luiza Steffens and Dinara Jaqueline Moura and Vanusa Manfredini and Carmen Regla Vargas},
  journal={Toxicology in vitro : an international journal published in association with BIBRA},
Maple syrup urine disease (MSUD) is an inherited deficiency of the branched-chain α-keto dehydrogenase complex, characterized by accumulation of the branched-chain amino acids (BCAAs) and their respective branched chain α-keto-acids (BCKAs), as well as by the presence of alloisoleucine (Allo). Studies have shown that oxidative stress is involved in the pathophysiology of MSUD. In this work, we investigated using the comet assay whether Allo, BCAAs and BCKAs could induce in vitro DNA damage, as… Expand
2 Citations
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Prevention of DNA damage by L-carnitine induced by metabolites accumulated in maple syrup urine disease in human peripheral leukocytes in vitro.
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L-Carnitine supplementation decreases DNA damage in treated MSUD patients.
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Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease
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DNA damage in an animal model of maple syrup urine disease.
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Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect
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