[13C]Valine Metabolism in Methylmalonicacidemia Using Nuclear Magnetic Resonance: Propinonate As an Obligate Intermediate

Overview

Journal Proc Natl Acad Sci U S A

Specialty Science

Date 1975 Sep 1

PMID 1059159

Citations 9

Authors

K Tanaka

I M Armitage

H S Ramsdell

Y E HSIA

S R Lipsky

L E ROSENBERG

Affiliations

Soon will be listed here.

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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Tomczyk M, Olkowicz M, Slominska E, Smolenski R Metabolites. 2021; 11(8).

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Hypermethioninaemia and 3-hydroxyisobutyric aciduria in an apparently healthy baby.

Congdon P, Haigh D, Smith R, Green A, Pollitt R J Inherit Metab Dis. 1981; 4(2):79-80.

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Metabolism of methylmalonic acid in rats. Is methylmalonyl-coenzyme a racemase deficiency symptomatic in man?.

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Tracer studies of the interconversion of R- and S-methylmalonic semialdehydes in man.

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Excessive excretion of beta-alanine and of 3-hydroxypropionic, R- and S-3-aminoisobutyric, R- and S-3-hydroxyisobutyric and S-2-(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydes.

Pollitt R, Green A, Smith R J Inherit Metab Dis. 1985; 8(2):75-9.

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