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Related Experiment Videos

Urinary C6-C12 dicarboxylic acylcarnitines in Reye's syndrome.

B M Tracey1, K N Cheng, J Rosankiewicz

  • 1Perinatal and Child Health, MRC Clinical Research Centre, Harrow, Middlesex, UK.

Clinica Chimica Acta; International Journal of Clinical Chemistry
|June 30, 1988
PubMed
Summary

New urinary dicarboxylic acylcarnitines were found in a child with Reye's syndrome. These findings suggest a potential role for impaired fatty acid beta-oxidation in the syndrome's development.

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Area of Science:

  • Biochemistry
  • Metabolic Disorders
  • Pediatrics

Background:

  • Reye's syndrome is a severe condition affecting multiple organs, primarily the brain and liver.
  • The exact metabolic derangements in Reye's syndrome are not fully understood.
  • Acylcarnitines are important markers of fatty acid metabolism.

Purpose of the Study:

  • To identify and quantify novel acylcarnitine metabolites in a patient with Reye's syndrome.
  • To investigate potential metabolic pathways disrupted in Reye's syndrome.

Main Methods:

  • Urine sample analysis from a 2-year-old girl with Reye's syndrome.
  • Extraction of acylcarnitines using ion-exchange chromatography.
  • Analysis via high-resolution fast-atom-bombardment mass spectrometry and capillary gas chromatography.

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Main Results:

  • First-time identification of C6-C12 dicarboxylic acylcarnitines in urine.
  • Dodecandioylcarnitine was the most abundant, exceeding free dodecandioic acid levels.
  • Other dicarboxylic acylcarnitines (adipic, suberic, sebacic) were present at lower concentrations relative to their free acids.

Conclusions:

  • Impaired beta-oxidation of dicarboxylic acids may occur in Reye's syndrome.
  • Accumulation of dodecandioylcarnitine suggests a metabolic block.
  • These findings may contribute to understanding the pathogenesis of Reye's syndrome.