Identification of methyl–branched chain dicarboxylic acids in amniotic fluid and urine in propionic and methylmalonic acidemia

Cornelis Jakobs, Lambertus Dorland, Lawrence Sweetman, Marinus Duran, William L. Nyhan, Sybe K. Wadman

Research output: Contribution to journalArticlepeer-review

8 Scopus citations

Abstract

3-Methyladipic, 4-methylpimelic, 4-meth-ylsuberic, pimelic, and azeleic acids were identified by gas chromatography-mass spectrometry in the amniotic fluid of fetuses with propionic acidemia. These compounds were virtually undetectable in normal amniotic fluid. Concentrations much higher than those of the amniotic fluid were found in the urine of neonatal infants with propionic acidemia and methylmalonic acidemia. It appears that the accumulation of these compounds is a consequence of the accumulation of propionyl-CoA. Evidence was obtained for the presence of other methyl-branched chain dicarboxylic acids. 4-Methylpimelic acid and 4-methylsuberic acid have not previously been identified in human physiological fluids.

Original languageEnglish (US)
Pages (from-to)1185-1190
Number of pages6
JournalPediatric Research
Volume18
Issue number11
DOIs
StatePublished - Nov 1984
Externally publishedYes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Fingerprint Dive into the research topics of 'Identification of methyl–branched chain dicarboxylic acids in amniotic fluid and urine in propionic and methylmalonic acidemia'. Together they form a unique fingerprint.

Cite this