INHIBITION OF OXIDATIVE-PHOSPHORYLATION BY PALMITOYL-COA IN DIGITONIN-PERMEABILIZED FIBROBLASTS - IMPLICATIONS FOR LONG-CHAIN FATTY-ACID BETA-OXIDATION DISORDERS

被引：40

作者：

VENTURA, FV

RUITER, JPN

IJLST, L

ALMEIDA, IT

WANDERS, RJA

机构：

[1] UNIV HOSP AMSTERDAM,ACAD MED CTR,DEPT CLIN BIOCHEM,1105 AZ AMSTERDAM,NETHERLANDS

[2] UNIV LISBON,FAC FARM,CTR METAB & GENET,P-1699 LISBON,PORTUGAL

来源：

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE | 1995年 / 1272卷 / 01期

关键词：

FATTY ACID; MITOCHONDRION; FATTY ACID OXIDATION; INBORN ERROR;

D O I：

10.1016/0925-4439(95)00064-B

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：

Long-chain fatty acid oxidation deficient patients present early in life with more severe features than patients with a medium-chain fatty acid oxidation deficiency. This may be related to the more toxic effect of long-chain fatty acid derivatives. In this paper we have studied the effect of different acyl-CoA esters, and palmitoyl-CoA in particular, on succinate-driven oxidative phosphorylation, using digitonin permeabilized human fibroblasts. Palmitoyl-CoA was found to inhibit the succinate-driven oxidative phosphorylation in a concentration dependent manner. If the inhibition of the oxidative phosphorylation system is also expressed under in vivo conditions this might explain some of the abnormalities found in patients with defects in long-chain fatty acid beta-oxidation.

引用

页码：14 / 20

页数：7

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