FATTY-ACID BETA-OXIDATION IN LEUKOCYTES FROM CONTROL SUBJECTS AND MEDIUM-CHAIN ACYL-COA DEHYDROGENASE DEFICIENT PATIENTS

被引：19

作者：

WANDERS, RJA

IJLST, L

机构：

[1] Department of Pediatric Clinical Biochemistry, University Hospital Amsterdam, Amsterdam

来源：

BIOCHIMICA ET BIOPHYSICA ACTA | 1992年 / 1138卷 / 01期

关键词：

FATTY ACID; FATTY ACID OXIDATION; INBORN ERROR; MEDIUM CHAIN ACYL-COA DEHYDROGENASE;

D O I：

10.1016/0925-4439(92)90155-G

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

In recent years an increasing number of inherited diseases in man have been identified in which there is an impairment in mitochondrial fatty acid oxidation. Diagnosis is usually done by gas-chromatographic analysis of urine, which may give difficulties, since urinary abnormalities may only be present intermittently. We therefore studied whether leukocytes could be used to study mitochondrial beta-oxidation directly. The results described herein show that leukocytes are able to beta-oxidize octanoate and palmitate. Furthermore, clear abnormalities in octanoate beta-oxidation were found in leukocytes from patients with an established deficiency of medium-chain acyl-CoA dehydrogenase, suggesting that measurement of octanoate and palmitate beta-oxidation in leukocytes may contribute to rapid diagnosis of medium-chain acyl-CoA dehydrogenase deficiency and presumably other mitochondrial beta-oxidation disorders.

引用

页码：80 / 84

页数：5

共 23 条

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