Abstract:
: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.
journal_name
FEBS Lettjournal_title
FEBS lettersauthors
Ramazzotti A,Vanmansart V,Foury Fdoi
10.1016/s0014-5793(03)01498-4keywords:
subject
Has Abstractpub_date
2004-01-16 00:00:00pages
215-20issue
1-3eissn
0014-5793issn
1873-3468pii
S0014579303014984journal_volume
557pub_type
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