Regulation of Mitochondrial Iron Accumulation by Yfh1p, a Putative Homolog of Frataxin

• DOI: 10.1126/science.276.5319.1709
• Publication Date: 2002-07-27T09:45:50Z
• Authors: Michael Babcock; Deepika de Silva; Robert Oaks; Sandra Davis-Kaplan; Sarn Jiralerspong; Laura Montermini; Massimo Pandolfo; Jerry Kaplan
• Keywords: Frataxin

The gene responsible for Friedreich's ataxia, a disease characterized by neurodegeneration and cardiomyopathy, has recently been cloned its product designated frataxin. A in Saccharomyces cerevisiae was whose predicted protein high sequence similarity to the human frataxin protein. yeast (yeast homolog, YFH1) encodes mitochondrial involved iron homeostasis respiratory function. Human also shown be Characterizing mechanism which YFH1 regulates may help define pathologic process leading cell damage ataxia.