Abstract
Two boys, who are not related, with hypoglycemia and C 6-C 10-dicarboxylic aciduria were investigated. Besides substantial amounts of adipic, suberic and sebacic acids, the urinary metabolic profile of organic acids contained 5-OH-caproic acid and caproylglycine. During acute attacks the concentrations of adipic, suberic and sebacic acids were 300–530, 160–200 and 35–200 μg/mg creatinine, respectively, and the excretions of 5-OH-caproic acid and caproylglycine were 75–330 and 41–260 μg/mg creatinine, respectively. It is argued that the biosynthesis of adipic acid passes through an ω-oxidation, that the production of 5-OH-caproic acid is caused by an ω-1-oxidation, and that caproylglycine formation passes through a glycine N-acylase catalysed conjugation of accumulated caproic acid in the patients. Suberic acid and sebacic acid are in the same way ω-oxidation products of accumulated caprylic acid and capric acid, respectively. From the excretion pattern presented it is hypothesized that the patients suffer from a defect in the dehydrogenation of fatty acids in the β-oxidation pathway. The biological significance of the findings is discussed.
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