Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin

被引：770

作者：

Babcock, M

deSilva, D

Oaks, R

DavisKaplan, S

Jiralerspong, S

Montermini, L

Pandolfo, M

Kaplan, J

机构：

[1] UNIV UTAH, SCH MED, DEPT PATHOL, DIV CELL BIOL & IMMUNOL, SALT LAKE CITY, UT 84132 USA

[2] CTR RECH LOUIS CHARLES SIMARD, MONTREAL, PQ H2L 4M1, CANADA

[3] UNIV MONTREAL, DEPT MED, MONTREAL, PQ H2L 4M1, CANADA

[4] MCGILL UNIV, DEPT NEUROL & NEUROSURG, MONTREAL, PQ H3A 2B4, CANADA

来源：

SCIENCE | 1997年 / 276卷 / 5319期

关键词：

D O I：

10.1126/science.276.5319.1709

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

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.

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页码：1709 / 1712

页数：4

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