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Identification of 3‐hydroxy‐3‐ethylglutaric acid in urine of patients with propionic acidaemia
Author(s) -
Kuhara Tomiko,
Inoue Yoshito,
Shinka Toshihiro,
Matsumoto Isamuu,
Matsuo Masafumi
Publication year - 1983
Publication title -
biomedical mass spectrometry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.475
H-Index - 121
eISSN - 1096-9888
pISSN - 0306-042X
DOI - 10.1002/bms.1200101202
Subject(s) - urine , ketone bodies , ketone , chemistry , chromatography , gas chromatography–mass spectrometry , pyruvate carboxylase , biochemistry , organic chemistry , metabolism , enzyme , mass spectrometry
By gas chromatographic mass spectrometric analysis, 3‐hydroxy‐3‐ethylglutaric acid was identified in the urine of patients with propionyl CoA carboxylase deficiency. Simultaneously, large amounts of 3‐hydroxyvaleric acid and 3‐oxovaleric acid, as well as a number of metabolites previously well known in this disorder, were also found in the urine of the patients. It is suggested that formation of 3‐oxovaleric acid and 3‐hydroxyvaleric acid proceeds via 3‐hydroxy‐3‐ethylglutaryl CoA as an intermediate by a mechanism similar to that of ketone body formation.