Mass spectrometric identification of 2-hydroxydodecanedioic acid and its homologues in urine from patients with hopantenate therapy during clinical episode.
Urine from patients with calcium-4-(2,4-dihydroxy-3,3-dimethylbutyramido) butyrate hemihydrate (hopantenate) therapy during episodes of Reye's-like syndrome was found to contain a number of unusual dicarboxylic acids in high concentrations; odd- and even-numbered medium-chain dicarboxylic acids, alpha-hydroxydicarboxylic acids and beta-hydroxydicarboxylic acids. The abnormal excretion of dicarboxylic acids, alpha- and beta-hydroxydicarboxylic acids disappeared after discontinuance of hopantenate therapy. Besides the excretion of 2-hydroxydecandedioic acid, which has been previously described in Zellweger syndrome or neonatal adrenoleukodystrophy, a series of alpha-hydroxydicarboxylic acids was detected and identified. In this paper, we have characterized some new compounds by gas chromatography/mass spectrometry: 2-hydroxydodecanedioic acid, 2-hydroxydodecenedioic acid, 2-hydroxytetradecanedioic acid, 2-hydroxytetradecenedioic acid and 2-hydroxyoctanedioic acid.
Matsumoto
M
M
Division of Human Genetics, Kanazawa Medical University, Ishikawa, Japan.
Kuhara
T
T
Inoue
Y
Y
Shinka
T
T
Matsumoto
I
I
Kajita
M
M
D000368:Aged; D002675:Child, Preschool; D003998:Dicarboxylic Acids / Q000652:urine*; D005260:Female; D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D006880:Hydroxy Acids / Q000652:urine*; D008297:Male; D013058:Mass Spectrometry; D010205:Pantothenic Acid / Q000031:analogs & derivatives* / Q000378:metabolism / Q000627:therapeutic use; D012202:Reye Syndrome / Q000188:drug therapy; D005680:gamma-Aminobutyric Acid / Q000031:analogs & derivatives* / Q000378:metabolism / Q000627:therapeutic use
D003998:Dicarboxylic Acids; D006880:Hydroxy Acids; C016030:pantogab; D010205:Pantothenic Acid; D005680:gamma-Aminobutyric Acid; C063884:2-hydroxydodecanedioic acid