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Novel glycine conjugates in medium-chain acyl-CoA dehydrogenase deficiency.

Journal of inherited metabolic disease (1993-01-01)
J J Pitt
ABSTRACT

The glycine conjugates of isocaproic, 4-methylhexanoic, 7-hydroxyoctanoic and 8-hydroxyoctanoic acids have been identified in the urine of children with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency using gas chromatography-mass spectrometry of the trimethylsilyl derivatives. A quantitative study showed that the glycine conjugates of isocaproic and 4-methylhexanoics acids were excreted during acute episodes and in smaller amounts when subjects were asymptomatic. The glycine conjugates of 7-hydroxyoctanoic and 8-hydroxyoctanoic acids were detectable during acute episodes. None of the conjugates was detected in controls or controls receiving a diet containing medium-chain triglycerides. It is suggested that the glycine conjugates of isocaproic acid and 4-methylhexanoic acid are metabolites of branched-chain fatty acids and that they are specific for MCAD deficiency.

MATERIALS
Product Number
Brand
Product Description

Sigma-Aldrich
4-Methylvaleric acid, 99%
Sigma-Aldrich
4-Methylpentanoic acid, ≥98%, FCC, FG
Sigma-Aldrich
8-Hydroxyoctanoic acid, 98% (T)
Sigma-Aldrich
4-Methylhexanoic acid, 97%

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