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

K. Tanaka; Ian M Armitage; H. S. Ramsdell

doi:10.1073/pnas.72.9.3692

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

K. Tanaka
, Ian M Armitage
, H. S. Ramsdell

Research output: Contribution to journal › Article › peer-review

45 Scopus citations

Abstract

[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.

Original language	English (US)
Pages (from-to)	3692-3696
Number of pages	5
Journal	Proceedings of the National Academy of Sciences of the United States of America
Volume	72
Issue number	9
DOIs	https://doi.org/10.1073/pnas.72.9.3692
State	Published - 1975
Externally published	Yes

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10.1073/pnas.72.9.3692

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title = "[13C]valine metabolism in methylmalonicacidemia using nuclear magnetic resonance: propionate as an obligate intermediate",

abstract = "[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.",

author = "K. Tanaka and Armitage, \{Ian M\} and Ramsdell, \{H. S.\}",

year = "1975",

doi = "10.1073/pnas.72.9.3692",

language = "English (US)",

volume = "72",

pages = "3692--3696",

journal = "Proceedings of the National Academy of Sciences of the United States of America",

issn = "0027-8424",

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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.

UR - https://www.scopus.com/pages/publications/0016554407

UR - https://www.scopus.com/pages/publications/0016554407#tab=citedBy

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 -

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

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