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Gene Review

AAC1  -  ADP/ATP carrier protein AAC1

Saccharomyces cerevisiae S288c

Synonyms: ADP,ATP carrier protein 1, ADP/ATP translocase 1, ANT 1, Adenine nucleotide translocator 1, YM9796.09C, ...
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Disease relevance of AAC1


High impact information on AAC1

  • Vpr favors the permeabilization of artificial membranes containing the purified PTPC or defined PTPC components such as the adenine nucleotide translocator (ANT) combined with Bax [2].
  • ANT-1 is a component of the mitochondrial permeability transition complex, a protein aggregate connecting the inner with the outer mitochondrial membrane that has recently been implicated in apoptosis [3].
  • Dominant missense mutations were found in the gene encoding the heart and skeletal muscle-specific isoform of the adenine nucleotide translocator (ANT1) in families with autosomal dominant progressive external opthalmoplegia and in a sporadic patient [1].
  • The newly discovered gene, AAC3, is homologous with the previously known genes AAC1 and AAC2 [4].
  • In reconstitution studies, the rate of nucleotide transport by isolated AAC1 protein is approximately 40% that of the AAC2 protein [5].

Biological context of AAC1

  • All three genes (AAC1, AAC2 and AAC3) encoding the mitochondrial ADP/ATP translocator, were inactivated in a haploid yeast strain by a gene disruption technique [6].
  • In this work, we analysed the 5' upstream region of AAC1 by sequencing and by mapping the transcription initiation site of the gene [7].
  • The data presented show that AAC1 expression, while constitutive under all aerobic conditions tested, is repressed during anaerobiosis in a heme-independent manner [7].
  • The Klaac null mutation, which causes a respiratory-deficient phenotype, was fully complemented by AAC2, the Saccharomyces cerevisiae major gene for the ADP/ATP carrier and also by AAC1, a gene that is poorly expressed in S. cerevisiae [8].
  • AAC2 contains a 954-base pair open reading frame coding for a protein of 318 amino acids which is highly homologous to the AAC1 gene product except that it is nine amino acids longer at the NH2 terminus [9].

Anatomical context of AAC1

  • As demonstrated by measurements of [14C]ADP specific binding and transport, all three translocator proteins, AAC1, AAC2 and AAC3 when present in the mitochondrial membrane, exhibited similar translocation properties [10].
  • Structure-function studies of adenine nucleotide transport in mitochondria. II. Biochemical analysis of distinct AAC1 and AAC2 proteins in yeast [5].
  • The ADP-ATP carrier (also referred to as the adenine nucleotide translocator) of Saccharomyces cerevisiae is encoded by a nuclear gene, translated in the cytosol, and imported into the mitochondrial inner membrane [11].
  • The consequence of the complexity of the metabolic network on the amount of control strength of adenine nucleotide translocator was investigated with isolated rat liver mitochondria [12].

Associations of AAC1 with chemical compounds


Other interactions of AAC1


Analytical, diagnostic and therapeutic context of AAC1

  • Sequence analysis of the ANT2 proximal promoter in comparison to that of the third yeast adenine nucleotide translocator (AAC3) led us to identify a new motif termed GRBOX [18].
  • The functional relationship between the adenine nucleotide translocator (ANT) and the mitochondrial multiple conductance channel (MCC) was investigated using patch-clamp techniques [19].


  1. Complete loss-of-function of the heart/muscle-specific adenine nucleotide translocator is associated with mitochondrial myopathy and cardiomyopathy. Palmieri, L., Alberio, S., Pisano, I., Lodi, T., Meznaric-Petrusa, M., Zidar, J., Santoro, A., Scarcia, P., Fontanesi, F., Lamantea, E., Ferrero, I., Zeviani, M. Hum. Mol. Genet. (2005) [Pubmed]
  2. The HIV-1 viral protein R induces apoptosis via a direct effect on the mitochondrial permeability transition pore. Jacotot, E., Ravagnan, L., Loeffler, M., Ferri, K.F., Vieira, H.L., Zamzami, N., Costantini, P., Druillennec, S., Hoebeke, J., Briand, J.P., Irinopoulou, T., Daugas, E., Susin, S.A., Cointe, D., Xie, Z.H., Reed, J.C., Roques, B.P., Kroemer, G. J. Exp. Med. (2000) [Pubmed]
  3. Adenine nucleotide translocase-1, a component of the permeability transition pore, can dominantly induce apoptosis. Bauer, M.K., Schubert, A., Rocks, O., Grimm, S. J. Cell Biol. (1999) [Pubmed]
  4. A third ADP/ATP translocator gene in yeast. Kolarov, J., Kolarova, N., Nelson, N. J. Biol. Chem. (1990) [Pubmed]
  5. Structure-function studies of adenine nucleotide transport in mitochondria. II. Biochemical analysis of distinct AAC1 and AAC2 proteins in yeast. Gawaz, M., Douglas, M.G., Klingenberg, M. J. Biol. Chem. (1990) [Pubmed]
  6. ADP/ATP translocator is essential only for anaerobic growth of yeast Saccharomyces cerevisiae. Drgon, T., Sabová, L., Nelson, N., Kolarov, J. FEBS Lett. (1991) [Pubmed]
  7. Transcription of the AAC1 gene encoding an isoform of mitochondrial ADP/ATP carrier in Saccharomyces cerevisiae is regulated by oxygen in a heme-independent manner. Gavurníková, G., Sabova, L., Kissová, I., Haviernik, P., Kolarov, J. Eur. J. Biochem. (1996) [Pubmed]
  8. Heterologous complementation of the Klaac null mutation of Kluyveromyces lactis by the Saccharomyces cerevisiae AAC3 gene encoding the ADP/ATP carrier. Fontanesi, F., Viola, A.M., Ferrero, I. FEMS Yeast Res. (2006) [Pubmed]
  9. Separate genes encode functionally equivalent ADP/ATP carrier proteins in Saccharomyces cerevisiae. Isolation and analysis of AAC2. Lawson, J.E., Douglas, M.G. J. Biol. Chem. (1988) [Pubmed]
  10. Yeast ADP/ATP carrier (AAC) proteins exhibit similar enzymatic properties but their deletion produces different phenotypes. Drgon, T., Sabová, L., Gavurniková, G., Kolarov, J. FEBS Lett. (1992) [Pubmed]
  11. ADP-ATP carrier of Saccharomyces cerevisiae contains a mitochondrial import signal between amino acids 72 and 111. Smagula, C.S., Douglas, M.G. J. Cell. Biochem. (1988) [Pubmed]
  12. Control of mitochondrial respiration. The contribution of the adenine nucleotide translocator depends on the ATP- and ADP-consuming enzymes. Gellerich, F.N., Bohnensack, R., Kunz, W. Biochim. Biophys. Acta (1983) [Pubmed]
  13. A Klaac null mutant of Kluyveromyces lactis is complemented by a single copy of the Saccharomyces cerevisiae AAC1 gene. Viola, A.M., Lodi, T., Ferrero, I. Curr. Genet. (1999) [Pubmed]
  14. Doxorubicin, daunorubicin, and mitoxantrone cytotoxicity in yeast. Kule, C., Ondrejickova, O., Verner, K. Mol. Pharmacol. (1994) [Pubmed]
  15. The isolation and reconstitution of the ADP/ATP carrier from wild-type Saccharomyces cerevisiae. Identification of primarily one type (AAC-2). Knirsch, M., Gawaz, M.P., Klingenberg, M. FEBS Lett. (1989) [Pubmed]
  16. Dynamic regulation of yeast glycolytic oscillations by mitochondrial functions. Aon, M.A., Cortassa, S., Westerhoff, H.V., Berden, J.A., Van Spronsen, E., Van Dam, K. J. Cell. Sci. (1991) [Pubmed]
  17. Deletion of amino acids 261-269 in the brown fat uncoupling protein converts the carrier into a pore. González-Barroso, M.M., Fleury, C., Levi-Meyrueis, C., Zaragoza, P., Bouillaud, F., Rial, E. Biochemistry (1997) [Pubmed]
  18. Expression of human ANT2 gene in highly proliferative cells: GRBOX, a new transcriptional element, is involved in the regulation of glycolytic ATP import into mitochondria. Giraud, S., Bonod-Bidaud, C., Wesolowski-Louvel, M., Stepien, G. J. Mol. Biol. (1998) [Pubmed]
  19. Activity of the mitochondrial multiple conductance channel is independent of the adenine nucleotide translocator. Lohret, T.A., Murphy, R.C., Drgoñ, T., Kinnally, K.W. J. Biol. Chem. (1996) [Pubmed]
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