3-Hydroxypropionate: Significance of β-Oxidation of Propionate in Patients with Propionic Acidemia and Methylmalonic Acidemia

[l-(14)C]Propionate administered intravenously was metabolized to methylmalonate, to 3-hydroxypropionate, and to methylcitrate in the urine of a patient with methylmalonic acidemia. L-[U-(14)C]Isoleucine and L-[U-(14)C]valine were also converted to urinary methylmalonate and to 3-hydroxypropionate i...

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Pubblicato in:	Proc Natl Acad Sci U S A
Autori principali:	Ando, Toshiyuki, Rasmussen, Karsten, Nyhan, William L., Hull, David
Natura:	Artigo
Lingua:	Inglês
Pubblicazione:	National Academy of Sciences 1972
Soggetti:	Biological Sciences: Medical Sciences
Accesso online:	https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC389649/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/4507604/ https://ncbi.nlm.nih.govhttps://doi.org/10.1073/pnas.69.10.2807
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