ADP/ATP translocase, adenine nucleotide translocase (ANT) or ADP/ATP carrier protein
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Function
- voltage-gated channel of inner mitochondrial membrane
- in association with VDAC-1 (outer mitochondrial membrane), ANT is thought to form the major pathway for movement of adenine nucleotides (ATP, ADP) through the outer mitochondrial membrane, the mitochondrial permeability transition pore
- opening of ANT dissipates the H+ gradient across the inner mitochondrial membrane, uncoupling oxidative phosphorylation
- may serve as the mitochondrial binding site for cyclophilin D
Pathology
- mutations in the gene for ANT-1 are associated with large deletions in mitochondrial DNA, possibly due to deficient adenine nucleotide pools for mitochondrial DNA synthesis or lack of a sufficient energy source for synthesis of mitochondrial DNA, or maintenance of its integrity
More general terms
- voltage-gated ion channel
- mitochondrial membrane protein
- oligomerizing protein
- carrier protein (transporter)
More specific terms
- ADP/ATP translocase-1 (adenine nucleotide translocator 1, ANT 1, ADP-ATP carrier protein 1, solute carrier family 25 member 4, heart/skeletal ADP/ATP carrier protein, SLC25A4, ANT1)
- ADP/ATP translocase-2 (adenine nucleotide translocator 2, ANT 2, ADP-ATP carrier protein 2, solute carrier family 25 member 5, fibroblast ADP/ATP carrier protein)
- ADP/ATP translocase-3 (solute carrier family 25 member 6, SLC25A6, liver ADP/ATP carrier protein)
- ADP/ATP translocase-3 (Y chromosome)
References
- ↑ Rottenberg H, Marbach M. Regulation of Ca2+ transport in brain mitochondria. I. The mechanism of spermine enhancement of Ca2+ uptake and retention. Biochim Biophys Acta. 1990 Mar 15;1016(1):77-86. PMID: https://www.ncbi.nlm.nih.gov/pubmed/2310743
Rottenberg H, Marbach M. Regulation of Ca2+ transport in brain mitochondria. II. The mechanism of the adenine nucleotides enhancement of Ca2+ uptake and retention. Biochim Biophys Acta. 1990 Mar 15;1016(1):87-98. PMID: https://www.ncbi.nlm.nih.gov/pubmed/2310744 - ↑ OMIM https://mirror.omim.org/entry/103220
- ↑ Moraes CT. A helicase is born. Nat Genet. 2001 Jul;28(3):200-1. PMID: https://www.ncbi.nlm.nih.gov/pubmed/11431681
Spelbrink JN, Li FY, Tiranti V, Nikali K, Yuan QP, et al Human mitochondrial DNA deletions associated with mutations in the gene encoding Twinkle, a phage T7 gene 4-like protein localized in mitochondria. Nat Genet. 2001 Jul;28(3):223-31. Erratum in: Nat Genet 2001 Sep;29(1):100. PMID: https://www.ncbi.nlm.nih.gov/pubmed/11431692 - ↑ Adams JM, Cory S. Life-or-death decisions by the Bcl-2 protein family. Trends Biochem Sci. 2001 Jan;26(1):61-6. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/11165519