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The identification of 3‐keto‐2‐methylvaleric acid and 3‐hydroxy‐2‐methylvaleric acid in a patient with propionic acidemia
Author(s) -
Truscott Roger J. W.,
Pullin Christopher J.,
Halpern Berthold,
Hammond Judy,
Haan Eric,
Danks David M.
Publication year - 1979
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.1200060706
Subject(s) - propionic acidemia , decarboxylation , urine , ketone , chemistry , ketone bodies , methylmalonic acidemia , chromatography , biochemistry , organic chemistry , medicine , metabolism , catalysis
Two abnormal metabolites, 3‐keto‐2‐methylvaleric acid and 3‐hydroxy‐2‐methylvaleric acid, have been identified and quantitated in the urine of a child with propionic acidemia. These metabolites may be produced as a result of the self‐condensation of propionyl‐CoA. Data are presented to show that the unusual ketone, 3‐pentanone, which has been observed previously in the urine of patients with propionic acidemia, is produced as a result of the decarboxylation of 3‐keto‐2‐methylvaleric acid.