期刊
FEBS LETTERS
卷 557, 期 1-3, 页码 215-220出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/S0014-5793(03)01498-4
关键词
Fe-S cluster; frataxin; ISU; synthetic lethal; yeast
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 crosslinking experiments in isolated mitochondria provide evidence for a direct interaction between Yfh1p and Isu1p. (C) 2003 Published by Elsevier B.V. on behalf of the Federation of European Biochemical Societies.
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