Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect.

@article{Mescka2013ProteinAL,
  title={Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect.},
  author={Caroline Paula Mescka and Carlos Alberto Yasin Wayhs and Camila Simioni Vanzin and Giovana Brondani Biancini and Gilian Guerreiro and Vanusa Manfredini and Carolina Souza and Moacir Wajner and Carlos Severo Dutra-Filho and Carmen Regla Vargas},
  journal={International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience},
  year={2013},
  volume={31 1},
  pages={21-4}
}
Maple syrup urine disease (MSUD) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids (BCAA) leucine, isoleucine, valine and the corresponding branched-chain α-keto acids. This disorder is clinically characterized by ketoacidosis, seizures, coma, psychomotor delay and mental retardation whose pathophysiology is not completely understood. Recent studies have shown that oxidative stress may be involved in neuropathology of MSUD. l… CONTINUE READING

4 Figures & Tables

Connections & Topics

Mentioned Connections BETA
Adenosine TriphosphateChemical or drug is product of biological processEnergy Metabolism
l - Carnitine ( l - Car ) plays a central role in the cellular energy metabolism because it transports long - chain fatty acids for oxidation and ATP generation .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
This disorder is clinically characterized by ketoacidosis , seizures , coma , psychomotor delay and mental retardation whose pathophysiology is not completely understood .
This disorder is clinically characterized by ketoacidosis , seizures , coma , psychomotor delay and mental retardation whose pathophysiology is not completely understood .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
LeucineNo subtypeValine
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
ValineNo subtypeLeucine
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
Maple syrup urine disease ( MSUD ) is an inborn error of metabolism biochemically characterized by elevated levels of the branched chain amino acids ( BCAA ) leucine , isoleucine , valine and the corresponding branched - chain α-keto acids .
All Topics