Deficiency of the iron-sulfur clusters of mitochondrial reduced nicotinamide-adenine dinucleotide-ubiquinone oxidoreductase (complex I) in an infant with congenital lactic acidosis.

We report the case of an infant with hypoglycemia, progressive lactic acidosis, an increased serum lactate/pyruvate ratio, and elevated plasma alanine, who had a moderate to profound decrease in the ability of mitochondria from four organs to oxidize pyruvate, malate plus glutamate, citrate, and oth...

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Enregistré dans:
Détails bibliographiques
Publié dans:J Clin Invest
Auteurs principaux: Moreadith, R W, Batshaw, M L, Ohnishi, T, Kerr, D, Knox, B, Jackson, D, Hruban, R, Olson, J, Reynafarje, B, Lehninger, A L
Format: Artigo
Langue:Inglês
Publié: American Society for Clinical Investigation 1984
Sujets:
Research Article
Accès en ligne:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC425222/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/6432847/
https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI111484
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