Branched Chain Amino Acids Induce Apoptosis in Neural Cells without Mitochondrial Membrane Depolarization or Cytochrome c Release: Implications for Neurological Impairment Associated with Maple Syrup Urine Disease

Maple syrup urine disease (MSUD) is an inborn error of metabolism caused by a deficiency in branched chain α-keto acid dehydrogenase that can result in neurodegenerative sequelae in human infants. In the present study, increased concentrations of MSUD metabolites, in particular α-keto isocaproic aci...

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Main Authors: Jouvet, Philippe, Rustin, Pierre, Taylor, Deanna L., Pocock, Jennifer M., Felderhoff-Mueser, Ursula, Mazarakis, Nicholas D., Sarraf, Catherine, Joashi, Umesh, Kozma, Mary, Greenwood, Kirsty, Edwards, A. David, Mehmet, Huseyin
Formato: Artigo
Idioma:Inglês
Publicado em: The American Society for Cell Biology 2000
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC14893/
https://ncbi.nlm.nih.gov/pubmed/10793161
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