Identification of a human mitochondrial ABC transporter, the functional orthologue of yeast Atm1p

FEBS Letters

Lill, Roland¹  Kispal, Gyula¹  Csere, Peter¹ 

[1] Institut für Zytobiologie und Zytopathologie der Philipps-Universität Marburg, Robert-Koch-Str. 5, 35033 Marburg, Germany

关键词: ABC transporter;    Mitochondrion;    Iron;    Cytochrome;    Sideroblastic anemia;   

DOI  :  10.1016/S0014-5793(98)01560-9

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

We have sequenced the entire coding region of the human ABC transporter ABC7. The protein represents a ‘half-transporter' and displays high sequence similarity to the mouse ABC7 protein and to the mitochondrial ABC transporter Atm1p of Saccharomyces cerevisiae. As shown by immunostaining using a specific antibody, the human ABC7 protein (hABC7) is a constituent of mitochondria. The N-terminus of hABC7 contains the information for targeting and import into the organelles. When synthesised in yeast cells defective in Atm1p (strain Δatm1/hABC7), hABC7 protein can revert the strong growth defect observed for Δatm1 cells to near wild-type behaviour. The known phenotypical consequences of inactivation of the ATM1 gene are almost fully amended by expression of hABC7 protein. Δatm1/hABC7 cells harbour wild-type levels of cytochromes and extra-mitochondrial heme-containing proteins, they contain normal levels of mitochondrial iron, and the cellular content of glutathione is substantially reduced relative to the high levels detected in Δatm1 cells. Our results suggest that hABC7 is a mitochondrial protein, and represents the functional orthologue of yeast Atm1p.

【 授权许可】

Unknown