title: Mitochondrial functional interactions between frataxin and Isu1p, the iron-sulfur cluster scaffold protein, in Saccharomyces cerevisiae.
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.
authors.affiliation: Unité de Biochimie Physiologique, Croix du Sud, 2-20, 1348 Louvain-la-Neuve, Belgium.
authors.forename: Anna
authors.initials: A
authors.lastname: Ramazzotti
authors.forename: Vincent
authors.initials: V
authors.lastname: Vanmansart
authors.forename: Françoise
authors.initials: F
authors.lastname: Foury
mesh_terms: D005288:Ferredoxins / Q000378:metabolism*; D005621:Friedreich Ataxia / Q000235:genetics; D006801:Humans; D033862:Iron-Binding Proteins / Q000235:genetics / Q000378:metabolism*; D007506:Iron-Sulfur Proteins / Q000378:metabolism; D024101:Mitochondrial Proteins; D012441:Saccharomyces cerevisiae / Q000235:genetics / Q000378:metabolism*; D029701:Saccharomyces cerevisiae Proteins / Q000378:metabolism*; D000096923:Frataxin
chemical_list: D005288:Ferredoxins; D033862:Iron-Binding Proteins; D007506:Iron-Sulfur Proteins; D024101:Mitochondrial Proteins; D029701:Saccharomyces cerevisiae Proteins; D000096923:Frataxin; C121520:ISU1 protein, S cerevisiae