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

[α -13C]- and [α ,β -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 by13C nucle...

Full description

Saved in:
Bibliographic Details
Published inProceedings of the National Academy of Sciences - PNAS Vol. 72; no. 9; pp. 3692 - 3696
Main Authors Tanaka, Kay, Armitage, Ian M., Ramsdell, Howard S., Hsia, Y. Edward, Lipsky, Seymour R., Rosenberg, Leon E.
Format Journal Article
LanguageEnglish
Published National Academy of Sciences of the United States of America 01.01.1975
Subjects
Acetates
Atoms
Biochemistry
Crystals
Impurities
Metabolism
Noise spectra
Nuclear magnetic resonance
Propionates
Urine
Online AccessGet full text

Cover

More Information
Summary:[α -13C]- and [α ,β -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 by13C 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.
ISSN:0027-8424
1091-6490
DOI:10.1073/pnas.72.9.3692