Elsevier

Clinica Chimica Acta

Volume 126, Issue 1, 24 November 1982, Pages 53-67
Clinica Chimica Acta

In vitro fibroblast studies in a patient with C6-C10-dicarboxylic aciduria: evidence for a defect in general acyl-CoA dehydrogenase

https://doi.org/10.1016/0009-8981(82)90361-8Get rights and content

Abstract

Fatty acid oxidation studies were performed on cultured fibroblasts from a patients who had suffered from a Reye's syndrome-like episode, and who excreted adipic acid, suberic acid, sebacic acid, 5-OH-hexanoic acid, and hexanoylglycine. Assays using fatty acids of different chain length on both intact and disrupted cells indicated that the patient had a defect of the general (medium-chain) acyl-CoA dehydrogenase apoenzyme.

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