Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria

FEBS Letters

Cazzalini, Ornella¹  Foury, Françoise¹ 

[1] Unité de Biochimie Physiologique, Place Croix du Sud 2-20, 1348-Louvain-La-Neuve, Belgium

关键词: Friedreich's ataxia;    Yeast homologue;    Gene disruption;    Mitochondria;    Iron;    Copper;    ABC;    ATP-binding cassette;    mtDNA;    mitochondrial DNA;    PCR;    polymerase chain reaction;   

DOI  :  10.1016/S0014-5793(97)00734-5

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

Deletion of YDL120, the yeast homologue of the human gene responsible for Friedreich's ataxia, elicits decreased cellular respiration associated with decreased cytochrome c oxidase activity and, in certain nuclear backgrounds, mitochondrial DNA is lost. In the null mutants, the cellular growth is highly sensitive to oxidants, such as H₂O₂, iron and copper. However, only ferrous sulfate elicits loss of mitochondrial DNA. Mitochondria of the null mutants contain 10 times more iron than wild-type. The neurodegeneration observed in Friedreich's ataxia can be well explained on the basis of a mitochondrial iron overload responsible for an increased production of highly toxic free radicals.

【 授权许可】

Unknown