[13C]Valine metabolism in methylmalonicacidemia using nuclear magnetic resonance: propinonate as an obligate intermediate

[Alpha-13C]- and [alpha,beta-13C]valine were administered sequentially to a patient with methylmalonicacidemia to clarify the metabolic pathway of valine from methylmalonic acid semialdehyde to methylmalonyl-CoA. Methylmalonic acid was isolated from multiple urine samples, purified, and analyzed by...

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Published inProceedings of the National Academy of Sciences - PNAS Vol. 72; no. 9; pp. 3692 - 3696
Main Authors Tanaka, K, Armitage, I M, Ramsdell, H S, Hsia, Y E, Lipsky, S R, Rosenberg, L E
Format Journal Article
LanguageEnglish
Published United States National Acad Sciences 01.09.1975
Subjects
Amino Acid Metabolism, Inborn Errors - metabolism
Child
Coenzyme A - metabolism
Humans
Magnetic Resonance Spectroscopy
Male
Malonates - metabolism
Methylmalonic Acid - metabolism
Methylmalonic Acid - urine
Propionates - metabolism
Valine - metabolism
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Summary:[Alpha-13C]- and [alpha,beta-13C]valine were administered sequentially to a patient with methylmalonicacidemia to clarify the metabolic pathway of valine from methylmalonic acid semialdehyde to methylmalonyl-CoA. Methylmalonic acid was isolated from multiple urine samples, purified, and analyzed by 13C nuclear magnetic resonance spectroscopy. Contrary to the widely accepted view, the results show unequivocally that methylmalonic acid semialdehyde is decarboxylated to propionate before conversion to methylmalonyl CoA.
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ISSN:0027-8424
1091-6490
DOI:10.1073/pnas.72.9.3692