PMID: 6111361Oct 1, 1980Paper

Studies on the urinary acidic metabolites from three patients with methylmalonic aciduria

Biomedical Mass Spectrometry
T Kuhara, I Matsumoto

Abstract

By gas chromatographic mass spectrometric analysis, 2-methyl-3-oxovaleric acid, 3-oxovaleric acid, 3-hydroxyvaleric acid and 2-methyl-3-hydroxybutyric acid were identified in the urine from three patients with methylmalonic aciduria. The level of 2-methyl-3-oxovaleric acid increased with the increase of methylmalonic acid concentration and the level of 3-oxovaleric acid increased with the increase of ketone bodies. It was demonstrated that these metabolites are detectable from patients with propionic acidemia and methylmalonic aciduria when propionyl CoA accumulates in the cells of the patients either primarily or secondarily.

References

Oct 15, 1975·Clinica Chimica Acta; International Journal of Clinical Chemistry·S Landaas
Mar 1, 1978·Biomedical Mass Spectrometry·L SweetmanY Estrada
Oct 1, 1972·Proceedings of the National Academy of Sciences of the United States of America·T AndoD Hull
May 30, 1973·Clinica Chimica Acta; International Journal of Clinical Chemistry·O StokkeR Schnitler
Oct 1, 1967·Archives of Disease in Childhood·V G OberholzerW F Young

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Citations

Jan 1, 1982·Journal of Inherited Metabolic Disease·T KuharaM Matsuo
Aug 4, 1982·Clinica Chimica Acta; International Journal of Clinical Chemistry·T KuharaI Matsumoto
Jul 16, 1984·Clinica Chimica Acta; International Journal of Clinical Chemistry·C JakobsW L Nyhan
Oct 1, 1994·Critical Reviews in Oncology/hematology·A A QureshiB A Cooper
Jan 11, 2017·Lipids·Nikita Ikon, Robert O Ryan
Oct 26, 2000·Journal of Chromatography. B, Biomedical Sciences and Applications·C ZhangI Matsumoto

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