THE KINETIC BASIS OF THRESHOLD EFFECTS OBSERVED IN MITOCHONDRIAL DISEASES - A SYSTEMIC APPROACH

被引：116

作者：

LETELLIER, T

HEINRICH, R

MALGAT, M

MAZAT, JP

机构：

[1] UNIV BORDEAUX 2, F-33076 BORDEAUX, FRANCE

[2] HUMBOLDT UNIV BERLIN, INST BIOPHYS, FACHBEREICH BIOL, D-10115 BERLIN, GERMANY

来源：

BIOCHEMICAL JOURNAL | 1994年 / 302卷

关键词：

D O I：

10.1042/bj3020171

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：

Threshold effects in the expression of metabolic diseases have often been observed in mitochondrial pathologies i.e. the clinical demonstration of the disease appears only when the activity of a step has been reduced to a rather low level. We show experimentally that an inhibition of cytochrome c oxidase activity by cyanide, simulating a defect in this step, leads to a decrease in mitochondrial respiration which then exhibits a threshold behaviour similar to that observed in mitochondrial diseases. We discuss this behaviour in terms of metabolic control theory and construct a mathematical model simulating this behaviour.

引用

页码：171 / 174

页数：4

共 22 条

[1]

BINDOFF LA, 1990, THESIS NEWCASTLE UPO

[2]

CHANCE B, 1956, ADV ENZYMOL REL S BI, V17, P65

[3] MELAS MUTATION IN MTDNA BINDING-SITE FOR TRANSCRIPTION TERMINATION FACTOR CAUSES DEFECTS IN PROTEIN-SYNTHESIS AND IN RESPIRATION BUT NO CHANGE IN LEVELS OF UPSTREAM AND DOWNSTREAM MATURE TRANSCRIPTS [J].