Fatty acid oxidation in fibroblasts from patients with defects in β-oxidation and in the respiratory chain

被引：24

作者：

Venizelos, N ^{[1
]}

von Döbeln, U ^{[1
]}

Hagenfeldt, L ^{[1
]}

机构：

[1] Huddinge Univ Hosp, Karolinska Inst, Div Clin Chem, S-14186 Huddinge, Sweden

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 1998年 / 21卷 / 04期

关键词：

D O I：

10.1023/A:1005310809714

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Fatty acid oxidation has been studied with the tritium release assay in cultured fibroblasts from patients with defects in beta-oxidation and in the mitochondrial respiratory chain. Cells from all patients with beta-oxidation defects and cells from 10 of 16 patients with respiratory chain defects showed an impairment of fatty acid oxidation. The result of the tritium release assay is not only dependent on the proper function of the beta-oxidation cycle but is also influenced by the reoxidation of reduced cofactors. The assay can thus be used to study the expression of respiratory chain defects in cultured fibroblasts.

引用

页码：409 / 415

页数：7

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