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

Peter Csere, Roland Lill, Gyula Kispal

Research output: Contribution to journalArticle

115 Citations (Scopus)


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. Copyright (C) 1998 Federation of European Biochemical Societies.

Original languageEnglish
Pages (from-to)266-270
Number of pages5
JournalFEBS letters
Issue number2
Publication statusPublished - Dec 18 1998


  • ABC transporter
  • Cytochrome
  • Iron
  • Mitochondrion
  • Sideroblastic anemia

ASJC Scopus subject areas

  • Biophysics
  • Structural Biology
  • Biochemistry
  • Molecular Biology
  • Genetics
  • Cell Biology

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