STUDIES ON MITOCHONDRIAL OXIDATIVE-PHOSPHORYLATION IN PERMEABILIZED HUMAN SKIN FIBROBLASTS - APPLICATION TO MITOCHONDRIAL ENCEPHALOMYOPATHIES

被引：63

作者：

WANDERS, RJA ^{[1
]}

RUITER, JPN ^{[1
]}

WIJBURG, FA ^{[1
]}

机构：

[1] UNIV HOSP AMSTERDAM,DEPT PEDIAT,1105 AZ AMSTERDAM,NETHERLANDS

来源：

BIOCHIMICA ET BIOPHYSICA ACTA | 1993年 / 1181卷 / 03期

关键词：

MITOCHONDRIA; INBORN ERROR; LACTIC ACIDOSIS; RESPIRATORY CHAIN; OXIDATIVE PHOSPHORYLATION;

D O I：

10.1016/0925-4439(93)90024-U

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 this report we describe that the functional capacity of the mitochondrial oxidative phosphorylation system can be studied in cultured skin fibroblasts permeabilized with a limited amount of digitonin. By using a variety of different oxidizable substrates, information can be obtained on the functional activity of complex I, for instance, which is important since different diseases in man have recently been identified in which complex I is deficient. The method described may contribute to the biochemical characterization of patients suffering from one of a variety of encephalomyopathies.

引用

页码：219 / 222

页数：4

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