ADP/ATP translocase 1
Details
- Name
- ADP/ATP translocase 1
- Synonyms
- Adenine nucleotide translocator 1
- ADP,ATP carrier protein 1
- ADP,ATP carrier protein, heart/skeletal muscle isoform T1
- ANT 1
- ANT1
- Solute carrier family 25 member 4
- Gene Name
- SLC25A4
- Organism
- Humans
- Amino acid sequence
>lcl|BSEQ0010417|ADP/ATP translocase 1 MGDHAWSFLKDFLAGGVAAAVSKTAVAPIERVKLLLQVQHASKQISAEKQYKGIIDCVVR IPKEQGFLSFWRGNLANVIRYFPTQALNFAFKDKYKQLFLGGVDRHKQFWRYFAGNLASG GAAGATSLCFVYPLDFARTRLAADVGKGAAQREFHGLGDCIIKIFKSDGLRGLYQGFNVS VQGIIIYRAAYFGVYDTAKGMLPDPKNVHIFVSWMIAQSVTAVAGLVSYPFDTVRRRMMM QSGRKGADIMYTGTVDCWRKIAKDEGAKAFFKGAWSNVLRGMGGAFVLVLYDEIKKYV
- Number of residues
- 298
- Molecular Weight
- 33064.265
- Theoretical pI
- 10.27
- GO Classification
- Functionsadenine transmembrane transporter activityProcessesadenine transport / apoptotic mitochondrial changes / energy reserve metabolic process / generation of precursor metabolites and energy / mitochondrial genome maintenance / negative regulation of necroptotic process / regulation of insulin secretion / small molecule metabolic process / transport / viral processComponentsintegral component of plasma membrane / mitochondrial inner membrane / mitochondrion / myelin sheath / nucleus
- General Function
- Adenine transmembrane transporter activity
- Specific Function
- Catalyzes the exchange of cytoplasmic ADP with mitochondrial ATP across the mitochondrial inner membrane.
- Pfam Domain Function
- Mito_carr (PF00153)
- Transmembrane Regions
- 5-39 75-100 109-143 176-202 207-241 273-298
- Cellular Location
- Mitochondrion inner membrane
- Gene sequence
>lcl|BSEQ0010418|ADP/ATP translocase 1 (SLC25A4) ATGGGTGATCACGCTTGGAGCTTCCTAAAGGACTTCCTGGCCGGGGGCGTCGCCGCTGCC GTCTCCAAGACCGCGGTCGCCCCCATCGAGAGGGTCAAACTGCTGCTGCAGGTCCAGCAT GCCAGCAAACAGATCAGTGCTGAGAAGCAGTACAAAGGGATCATTGATTGTGTGGTGAGA ATCCCTAAGGAGCAGGGCTTCCTCTCCTTCTGGAGGGGTAACCTGGCCAACGTGATCCGT TACTTCCCCACCCAAGCTCTCAACTTCGCCTTCAAGGACAAGTACAAGCAGCTCTTCTTA GGGGGTGTGGATCGGCATAAGCAGTTCTGGCGCTACTTTGCTGGTAACCTGGCGTCCGGT GGGGCCGCTGGGGCCACCTCCCTTTGCTTTGTCTACCCGCTGGACTTTGCTAGGACCAGG TTGGCTGCTGATGTGGGCAAGGGCGCCGCCCAGCGTGAGTTCCATGGTCTGGGCGACTGT ATCATCAAGATCTTCAAGTCTGATGGCCTGAGGGGGCTCTACCAGGGTTTCAACGTCTCT GTCCAAGGCATCATTATCTATAGAGCTGCCTACTTCGGAGTCTATGATACTGCCAAGGGG ATGCTGCCTGACCCCAAGAACGTGCACATTTTTGTGAGCTGGATGATTGCCCAGAGTGTG ACGGCAGTCGCAGGGCTGGTGTCCTACCCCTTTGACACTGTTCGTCGTAGAATGATGATG CAGTCCGGCCGGAAAGGGGCCGATATTATGTACACGGGGACAGTTGACTGCTGGAGGAAG ATTGCAAAAGACGAAGGAGCCAAGGCCTTCTTCAAAGGTGCCTGGTCCAATGTGCTGAGA GGCATGGGCGGTGCTTTTGTATTGGTGTTGTATGATGAGATCAAAAAATATGTCTAA
- Chromosome Location
- 4
- Locus
- 4q35
- External Identifiers
Resource Link UniProtKB ID P12235 UniProtKB Entry Name ADT1_HUMAN GenBank Protein ID 339920 GenBank Gene ID J02966 GenAtlas ID SLC25A4 HGNC ID HGNC:10990 - General References
- Neckelmann N, Li K, Wade RP, Shuster R, Wallace DC: cDNA sequence of a human skeletal muscle ADP/ATP translocator: lack of a leader peptide, divergence from a fibroblast translocator cDNA, and coevolution with mitochondrial DNA genes. Proc Natl Acad Sci U S A. 1987 Nov;84(21):7580-4. [Article]
- Cozens AL, Runswick MJ, Walker JE: DNA sequences of two expressed nuclear genes for human mitochondrial ADP/ATP translocase. J Mol Biol. 1989 Mar 20;206(2):261-80. [Article]
- Li K, Warner CK, Hodge JA, Minoshima S, Kudoh J, Fukuyama R, Maekawa M, Shimizu Y, Shimizu N, Wallace DC: A human muscle adenine nucleotide translocator gene has four exons, is located on chromosome 4, and is differentially expressed. J Biol Chem. 1989 Aug 25;264(24):13998-4004. [Article]
- Wang W, Shen P, Thiyagarajan S, Lin S, Palm C, Horvath R, Klopstock T, Cutler D, Pique L, Schrijver I, Davis RW, Mindrinos M, Speed TP, Scharfe C: Identification of rare DNA variants in mitochondrial disorders with improved array-based sequencing. Nucleic Acids Res. 2011 Jan;39(1):44-58. doi: 10.1093/nar/gkq750. Epub 2010 Sep 15. [Article]
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- Houldsworth J, Attardi G: Two distinct genes for ADP/ATP translocase are expressed at the mRNA level in adult human liver. Proc Natl Acad Sci U S A. 1988 Jan;85(2):377-81. [Article]
- Deniaud A, Brenner C, Kroemer G: Mitochondrial membrane permeabilization by HIV-1 Vpr. Mitochondrion. 2004 Jul;4(2-3):223-33. [Article]
- Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [Article]
- Burkard TR, Planyavsky M, Kaupe I, Breitwieser FP, Burckstummer T, Bennett KL, Superti-Furga G, Colinge J: Initial characterization of the human central proteome. BMC Syst Biol. 2011 Jan 26;5:17. doi: 10.1186/1752-0509-5-17. [Article]
- Echaniz-Laguna A, Chassagne M, Ceresuela J, Rouvet I, Padet S, Acquaviva C, Nataf S, Vinzio S, Bozon D, Mousson de Camaret B: Complete loss of expression of the ANT1 gene causing cardiomyopathy and myopathy. J Med Genet. 2012 Feb;49(2):146-50. doi: 10.1136/jmedgenet-2011-100504. Epub 2011 Dec 20. [Article]
- Vaca Jacome AS, Rabilloud T, Schaeffer-Reiss C, Rompais M, Ayoub D, Lane L, Bairoch A, Van Dorsselaer A, Carapito C: N-terminome analysis of the human mitochondrial proteome. Proteomics. 2015 Jul;15(14):2519-24. doi: 10.1002/pmic.201400617. Epub 2015 Jun 8. [Article]
- Kaukonen J, Juselius JK, Tiranti V, Kyttala A, Zeviani M, Comi GP, Keranen S, Peltonen L, Suomalainen A: Role of adenine nucleotide translocator 1 in mtDNA maintenance. Science. 2000 Aug 4;289(5480):782-5. [Article]
- Napoli L, Bordoni A, Zeviani M, Hadjigeorgiou GM, Sciacco M, Tiranti V, Terentiou A, Moggio M, Papadimitriou A, Scarlato G, Comi GP: A novel missense adenine nucleotide translocator-1 gene mutation in a Greek adPEO family. Neurology. 2001 Dec 26;57(12):2295-8. [Article]
- Komaki H, Fukazawa T, Houzen H, Yoshida K, Nonaka I, Goto Y: A novel D104G mutation in the adenine nucleotide translocator 1 gene in autosomal dominant progressive external ophthalmoplegia patients with mitochondrial DNA with multiple deletions. Ann Neurol. 2002 May;51(5):645-8. [Article]
- Agostino A, Valletta L, Chinnery PF, Ferrari G, Carrara F, Taylor RW, Schaefer AM, Turnbull DM, Tiranti V, Zeviani M: Mutations of ANT1, Twinkle, and POLG1 in sporadic progressive external ophthalmoplegia (PEO). Neurology. 2003 Apr 22;60(8):1354-6. [Article]
- 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: Complete loss-of-function of the heart/muscle-specific adenine nucleotide translocator is associated with mitochondrial myopathy and cardiomyopathy. Hum Mol Genet. 2005 Oct 15;14(20):3079-88. Epub 2005 Sep 9. [Article]
- Deschauer M, Hudson G, Muller T, Taylor RW, Chinnery PF, Zierz S: A novel ANT1 gene mutation with probable germline mosaicism in autosomal dominant progressive external ophthalmoplegia. Neuromuscul Disord. 2005 Apr;15(4):311-5. Epub 2005 Jan 28. [Article]
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Drug Relations
- Drug Relations
DrugBank ID Name Drug group Pharmacological action? Actions Details DB00171 ATP investigational, nutraceutical unknown Details DB00720 Clodronic acid approved, investigational, vet_approved yes inhibitor Details DB02426 Carboxyatractyloside experimental unknown Details DB03429 Tetrastearoyl cardiolipin experimental unknown Details DB04178 Di-Stearoyl-3-Sn-Phosphatidylcholine experimental unknown Details DB01736 [3-(Dodecanoylamino)Propyl](Hydroxy)Dimethylammonium experimental unknown Details DB01077 Etidronic acid approved unknown inhibitor Details