TY - JOUR
T1 - [13C]valine metabolism in methylmalonicacidemia using nuclear magnetic resonance
T2 - propionate as an obligate intermediate
AU - Tanaka, K.
AU - Armitage, Ian M
AU - Ramsdell, H. S.
PY - 1975
Y1 - 1975
N2 - [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 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.
AB - [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 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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U2 - 10.1073/pnas.72.9.3692
DO - 10.1073/pnas.72.9.3692
M3 - Article
C2 - 1059159
AN - SCOPUS:0016554407
SN - 0027-8424
VL - 72
SP - 3692
EP - 3696
JO - Proceedings of the National Academy of Sciences of the United States of America
JF - Proceedings of the National Academy of Sciences of the United States of America
IS - 9
ER -