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Gene Review

MDL1  -  ATP-binding cassette permease MDL1

Saccharomyces cerevisiae S288c

Synonyms: ABC transporter MDL1, ATP-dependent permease MDL1, mitochondrial, L9470.3, Multidrug resistance-like protein 1, YLR188W
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Disease relevance of MDL1

  • We demonstrate that over-expression of MDL1 in Deltaatm1 cells results in a reduction of mitochondrial iron content, and decreased sensitivity to H(2)O(2) and transition metal toxicity [1].
  • To characterize the ATP hydrolysis cycle of Mdl1p, the nucleotide-binding domain (NBD) was overexpressed in Escherichia coli and purified to homogeneity [2].

High impact information on MDL1


Biological context of MDL1

  • MDL1 is a high copy suppressor of ATM1: evidence for a role in resistance to oxidative stress [1].
  • On the other hand, deletion of MDL1, coding for an ABC-transporter involved in peptide export from the matrix space, only had minor effects on nuclear gene expression [7].
  • The ATP hydrolysis cycle of the nucleotide-binding domain of the mitochondrial ATP-binding cassette transporter Mdl1p [2].
  • The ABC transporter Mdl1p, a structural and functional homologue of the transporter associated with antigen processing (TAP) plays an important role in intracellular peptide transport from the mitochondrial matrix of Saccharomyces cerevisiae [2].
  • These results are consistent with an activation of Mdl1 upon dissociation from the ATP synthase and suggest a link of peptide export from mitochondria to the activity of the F(1)F(0)-ATP synthase and the cellular energy metabolism [8].

Anatomical context of MDL1

  • 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 [9].

Associations of MDL1 with chemical compounds


Other interactions of MDL1

  • One of the genes we identified was MDL1, which like ATM1, encodes a mitochondrial inner membrane ABC transporter [1].
  • The encoded protein is similar to those encoded by MDL1 and MDL2, two MDR-like genes of Saccharomyces cerevisiae [10].


  1. MDL1 is a high copy suppressor of ATM1: evidence for a role in resistance to oxidative stress. Chloupková, M., LeBard, L.S., Koeller, D.M. J. Mol. Biol. (2003) [Pubmed]
  2. The ATP hydrolysis cycle of the nucleotide-binding domain of the mitochondrial ATP-binding cassette transporter Mdl1p. Janas, E., Hofacker, M., Chen, M., Gompf, S., van der Does, C., Tampé, R. J. Biol. Chem. (2003) [Pubmed]
  3. Role of the ABC transporter Mdl1 in peptide export from mitochondria. Young, L., Leonhard, K., Tatsuta, T., Trowsdale, J., Langer, T. Science (2001) [Pubmed]
  4. A new pathway for protein export in Saccharomyces cerevisiae. Cleves, A.E., Cooper, D.N., Barondes, S.H., Kelly, R.B. J. Cell Biol. (1996) [Pubmed]
  5. Multidrug resistance-like genes of Arabidopsis required for auxin transport and auxin-mediated development. Noh, B., Murphy, A.S., Spalding, E.P. Plant Cell (2001) [Pubmed]
  6. Structural and Functional Fingerprint of the Mitochondrial ATP-binding Cassette Transporter Mdl1 from Saccharomyces cerevisiae. Hofacker, M., Gompf, S., Zutz, A., Presenti, C., Haase, W., van der Does, C., Model, K., Tampé, R. J. Biol. Chem. (2007) [Pubmed]
  7. Evidence for a novel mitochondria-to-nucleus signalling pathway in respiring cells lacking i-AAA protease and the ABC-transporter Mdl1. Arnold, I., Wagner-Ecker, M., Ansorge, W., Langer, T. Gene (2006) [Pubmed]
  8. Reversible assembly of the ATP-binding cassette transporter Mdl1 with the F1F0-ATP synthase in mitochondria. Galluhn, D., Langer, T. J. Biol. Chem. (2004) [Pubmed]
  9. Mitochondrial ABC transporters. Lill, R., Kispal, G. Res. Microbiol. (2001) [Pubmed]
  10. Genes encoding multiple drug resistance-like proteins in Aspergillus fumigatus and Aspergillus flavus. Tobin, M.B., Peery, R.B., Skatrud, P.L. Gene (1997) [Pubmed]
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