Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins.

@article{Schwab2006SecondaryMD,
  title={Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins.},
  author={Marina A Schwab and Sven Wolfgang Sauer and Juergen Guenther Okun and Leo G. J. Nijtmans and Richard R J Rodenburg and Lambert P.W.J. van den Heuvel and Stefan Dr{\"o}se and Ulrich Brandt and Georg F. Hoffmann and Henk J ter Laak and Stefan Koelker and Jan A M Smeitink},
  journal={The Biochemical journal},
  year={2006},
  volume={398 1},
  pages={
          107-12
        }
}
Mitochondrial dysfunction during acute metabolic crises is considered an important pathomechanism in inherited disorders of propionate metabolism, i.e. propionic and methylmalonic acidurias. Biochemically, these disorders are characterized by accumulation of propionyl-CoA and metabolites of alternative propionate oxidation. In the present study, we demonstrate uncompetitive inhibition of PDHc (pyruvate dehydrogenase complex) by propionyl-CoA in purified porcine enzyme and in submitochondrial… CONTINUE READING

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