Mitochondrial ABC transporters

Roland Lill, Gyula Kispal

Research output: Contribution to journalArticle

57 Citations (Scopus)


In contrast to bacteria, mitochondria contain only a few ATP binding cassette (ABC) transporters in their inner membrane. The known mitochondrial ABC proteins fall into two major classes that, in the yeast Saccharomyces cerevisiae, are represented by the half-transporter Atm1p and the two closely homologous proteins Mdl1p and Mdl2p. In humans two Atm1p orthologues (ABC7 and MTABC3) and two proteins homologous to Mdl1/2p have been localized to mitochondria. The Atm1p-like proteins perform an important function in mitochondrial iron homeostasis and in the maturation of Fe/S proteins in the cytosol. Mutations in ABC7 are causative of hereditary X-linked sideroblastic anemia and cerebellar ataxia (XLSA/A). MTABC3 may be a candidate gene for the lethal neonatal syndrome. The function of the mitochondrial Mdl1/2p-like proteins is not clear at present with the notable exception of murine ABC-me that may transport intermediates of heme biosynthesis from the matrix to the cytosol in erythroid tissues.

Original languageEnglish
Pages (from-to)331-340
Number of pages10
JournalResearch in Microbiology
Issue number3-4
Publication statusPublished - Jan 1 2001



  • ABC transporters
  • Fe/S cluster
  • Fe/S protein
  • Leucine biosynthesis
  • Mitochondria

ASJC Scopus subject areas

  • Microbiology
  • Molecular Biology

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