Quantification of 3-methylglutaconic acid in urine, plasma, and amniotic fluid by isotope-dilution gas chromatography/mass spectrometry.
A method is described for quantification of the trace metabolite, 3-methylglutaconic acid, by isotope-dilution gas chromatography/mass spectrometry using synthetic 3-[2,4,6-13C3]methylglutaconic acid. Results are shown for quantification of 3-methylglutaconic acid in plasma, urine, cerebrospinal fluid and amniotic fluid for both normal controls and patients with different forms of 3-methylglutaconic aciduria. A simple method for the synthesis and purification of 3-[2,4,6-13C3]methylglutaconic acid is also described.
Kelley
R I
RI
Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205.
D000328:Adult; D000653:Amniotic Fluid / Q000737:chemistry; D002648:Child; D002675:Child, Preschool; D008401:Gas Chromatography-Mass Spectrometry / Q000379:methods*; D005977:Glutarates / Q000032:analysis* / Q000097:blood / Q000134:cerebrospinal fluid / Q000652:urine; D006801:Humans; D007223:Infant
D005977:Glutarates; C022034:3-methylglutaconic acid