L-carnitine therapy in isovaleric acidemia.

Isovaleric acidemia, resulting from isovaleryl-coenzyme A dehydrogenase deficiency, is associated with marked reduction of free carnitine in both plasma and urine. Fast atom bombardment-mass spectrometry, hydrolysis, and gas chromatography/mass spectrometry have unequivocally identified the existenc...

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Publié dans:	J Clin Invest
Auteurs principaux:	Roe, C R, Millington, D S, Maltby, D A, Kahler, S G, Bohan, T P
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/PMC425423/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/6549017/ https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI111657
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