Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin.
The gene responsible for Friedreich's ataxia, a disease characterized by neurodegeneration and cardiomyopathy, has recently been cloned and its product designated frataxin. A gene in Saccharomyces cerevisiae was characterized whose predicted protein product has high sequence similarity to the human frataxin protein. The yeast gene (yeast frataxin homolog, YFH1) encodes a mitochondrial protein involved in iron homeostasis and respiratory function. Human frataxin also was shown to be a mitochondrial protein. Characterizing the mechanism by which YFH1 regulates iron homeostasis in yeast may help to define the pathologic process leading to cell damage in Friedreich's ataxia.
Babcock
M
M
Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA.
de Silva
D
D
Oaks
R
R
Davis-Kaplan
S
S
Jiralerspong
S
S
Montermini
L
L
Pandolfo
M
M
Kaplan
J
J
D001692:Biological Transport; D002352:Carrier Proteins / Q000378:metabolism; D002462:Cell Membrane / Q000378:metabolism; D002570:Ceruloplasmin; D003600:Cytosol / Q000378:metabolism; D005621:Friedreich Ataxia / Q000378:metabolism; D005656:Fungal Proteins / Q000235:genetics / Q000378:metabolism*; D005800:Genes, Fungal; D005816:Genetic Complementation Test; D006706:Homeostasis; D006801:Humans; D007501:Iron / Q000378:metabolism*; D033862:Iron-Binding Proteins; D026901:Membrane Transport Proteins / Q000378:metabolism; D008928:Mitochondria / Q000378:metabolism*; D018384:Oxidative Stress; D010088:Oxidoreductases / Q000378:metabolism; D017853:Phosphotransferases (Alcohol Group Acceptor) / Q000378:metabolism; D011993:Recombinant Fusion Proteins / Q000378:metabolism; D012441:Saccharomyces cerevisiae / Q000235:genetics / Q000254:growth & development / Q000378:metabolism*; D029701:Saccharomyces cerevisiae Proteins; D014170:Transformation, Genetic; D000096923:Frataxin
D002352:Carrier Proteins; D002570:Ceruloplasmin; D005656:Fungal Proteins; D007501:Iron; D033862:Iron-Binding Proteins; D026901:Membrane Transport Proteins; D010088:Oxidoreductases; D017853:Phosphotransferases (Alcohol Group Acceptor); D011993:Recombinant Fusion Proteins; D029701:Saccharomyces cerevisiae Proteins; D000096923:Frataxin; C098723:FTR1 protein, S cerevisiae; C085513:FET3 protein, S cerevisiae