Mitochondrial functional interactions between frataxin and Isu1p, the iron–sulfur cluster scaffold protein, in Saccharomyces cerevisiae

FEBS Letters

Vanmansart, Vincent¹  Foury, Françoise¹  Ramazzotti, Anna¹ 

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

关键词: Fe–S cluster;    Frataxin;    ISU;    Synthetic lethal;    Yeast;   

DOI  :  10.1016/S0014-5793(03)01498-4

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

Friedreich's ataxia is caused by a deficit in the mitochondrial protein frataxin. The present work demonstrates that in vivo yeast frataxin Yfh1p and Isu1p, the mitochondrial scaffold protein for the Fe–S cluster assembly, have tightly linked biological functions, acting in concert to promote the Fe–S cluster assembly. A synthetic lethal screen on high iron media with the mild G107D yfh1 mutant has specifically identified Isu1p. Analysis of the cellular phenotypes resulting from pairwise combinations of yfh1 and isu1 mutations, and cross-linking experiments in isolated mitochondria provide evidence for a direct interaction between Yfh1p and Isu1p.

【 授权许可】

Unknown