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. 1975 Sep;72(9):3692–3696. doi: 10.1073/pnas.72.9.3692

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

K Tanaka, I M Armitage, H S Ramsdell, Y E Hsia, S R Lipsky, L E Rosenberg
PMCID: PMC433063  PMID: 1059159

Abstract

[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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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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