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NameATP-binding cassette sub-family C member 9
Synonyms
  • Sulfonylurea receptor 2
  • SUR2
Gene NameABCC9
OrganismHuman
Amino acid sequence
>lcl|BSEQ0037023|ATP-binding cassette sub-family C member 9
MSLSFCGNNISSYNINDGVLQNSCFVDALNLVPHVFLLFITFPILFIGWGSQSSKVQIHH
NTWLHFPGHNLRWILTFALLFVHVCEIAEGIVSDSRRESRHLHLFMPAVMGFVATTTSIV
YYHNIETSNFPKLLLALFLYWVMAFITKTIKLVKYCQSGLDISNLRFCITGMMVILNGLL
MAVEINVIRVRRYVFFMNPQKVKPPEDLQDLGVRFLQPFVNLLSKATYWWMNTLIISAHK
KPIDLKAIGKLPIAMRAVTNYVCLKDAYEEQKKKVADHPNRTPSIWLAMYRAFGRPILLS
STFRYLADLLGFAGPLCISGIVQRVNETQNGTNNTTGISETLSSKEFLENAYVLAVLLFL
ALILQRTFLQASYYVTIETGINLRGALLAMIYNKILRLSTSNLSMGEMTLGQINNLVAIE
TNQLMWFLFLCPNLWAMPVQIIMGVILLYNLLGSSALVGAAVIVLLAPIQYFIATKLAEA
QKSTLDYSTERLKKTNEILKGIKLLKLYAWEHIFCKSVEETRMKELSSLKTFALYTSLSI
FMNAAIPIAAVLATFVTHAYASGNNLKPAEAFASLSLFHILVTPLFLLSTVVRFAVKAII
SVQKLNEFLLSDEIGDDSWRTGESSLPFESCKKHTGVQPKTINRKQPGRYHLDSYEQSTR
RLRPAETEDIAIKVTNGYFSWGSGLATLSNIDIRIPTGQLTMIVGQVGCGKSSLLLAILG
EMQTLEGKVHWSNVNESEPSFEATRSRNRYSVAYAAQKPWLLNATVEENITFGSPFNKQR
YKAVTDACSLQPDIDLLPFGDQTEIGERGINLSGGQRQRICVARALYQNTNIVFLDDPFS
ALDIHLSDHLMQEGILKFLQDDKRTLVLVTHKLQYLTHADWIIAMKDGSVLREGTLKDIQ
TKDVELYEHWKTLMNRQDQELEKDMEADQTTLERKTLRRAMYSREAKAQMEDEDEEEEEE
EDEDDNMSTVMRLRTKMPWKTCWRYLTSGGFFLLILMIFSKLLKHSVIVAIDYWLATWTS
EYSINNTGKADQTYYVAGFSILCGAGIFLCLVTSLTVEWMGLTAAKNLHHNLLNKIILGP
IRFFDTTPLGLILNRFSADTNIIDQHIPPTLESLTRSTLLCLSAIGMISYATPVFLVALL
PLGVAFYFIQKYFRVASKDLQELDDSTQLPLLCHFSETAEGLTTIRAFRHETRFKQRMLE
LTDTNNIAYLFLSAANRWLEVRTDYLGACIVLTASIASISGSSNSGLVGLGLLYALTITN
YLNWVVRNLADLEVQMGAVKKVNSFLTMESENYEGTMDPSQVPEHWPQEGEIKIHDLCVR
YENNLKPVLKHVKAYIKPGQKVGICGRTGSGKSSLSLAFFRMVDIFDGKIVIDGIDISKL
PLHTLRSRLSIILQDPILFSGSIRFNLDPECKCTDDRLWEALEIAQLKNMVKSLPGGLDA
VVTEGGENFSVGQRQLFCLARAFVRKSSILIMDEATASIDMATENILQKVVMTAFADRTV
VTIAHRVSSIMDAGLVLVFSEGILVECDTVPNLLAHKNGLFSTLVMTNK
Number of residues1549
Molecular Weight174221.7
Theoretical pI7.39
GO Classification
Functions
  • ion channel binding
  • potassium channel regulator activity
  • transporter activity
  • ATP binding
  • potassium channel activity
  • sulfonylurea receptor activity
  • ATPase activity, coupled to transmembrane movement of substances
Processes
  • defense response to virus
  • signal transduction
  • potassium ion transport
  • synaptic transmission
  • transmembrane transport
  • potassium ion import
Components
  • ATP-sensitive potassium channel complex
  • sarcomere
  • plasma membrane
  • voltage-gated potassium channel complex
  • sarcolemma
General FunctionTransporter activity
Specific FunctionSubunit of ATP-sensitive potassium channels (KATP). Can form cardiac and smooth muscle-type KATP channels with KCNJ11. KCNJ11 forms the channel pore while ABCC9 is required for activation and regulation.
Pfam Domain Function
Transmembrane Regions31-51 73-93 102-122 133-153 168-188 302-322 351-371 424-444 456-476 532-552 572-592 991-1011 1035-1055 1128-1148 1246-1266
GenBank Protein ID3127176
UniProtKB IDO60706
UniProtKB Entry NameABCC9_HUMAN
Cellular LocationMembrane
Gene sequence
>lcl|BSEQ0016101|ATP-binding cassette sub-family C member 9 (ABCC9)
ATGAGCCTTTCATTTTGTGGTAACAACATTTCTTCATATAATATCAACGATGGTGTACTA
CAAAATTCCTGCTTTGTGGATGCCCTCAACCTGGTCCCTCATGTCTTTCTGTTGTTTATC
ACTTTTCCAATATTGTTTATTGGGTGGGGGAGCCAAAGCTCAAAAGTACAAATTCACCAC
AACACATGGCTTCATTTTCCGGGACATAACCTGAGATGGATTCTTACATTCGCTCTCCTG
TTTGTGCATGTCTGTGAAATAGCAGAAGGCATTGTTTCAGACTCGCGGCGGGAATCAAGG
CACCTCCACCTCTTTATGCCAGCCGTGATGGGATTCGTTGCCACTACAACATCGATAGTG
TATTATCATAATATCGAAACATCAAATTTTCCTAAATTACTTTTAGCCCTGTTCCTGTAT
TGGGTAATGGCCTTTATTACAAAAACAATAAAATTGGTTAAGTACTGTCAGTCTGGCTTG
GACATATCAAACCTGCGTTTCTGCATCACAGGCATGATGGTCATCTTGAATGGGCTCTTG
ATGGCTGTGGAGATCAATGTCATTCGAGTCAGGAGATATGTATTTTTCATGAATCCTCAG
AAAGTAAAGCCTCCTGAAGACCTCCAGGATCTGGGAGTGAGATTTCTTCAACCATTTGTG
AATTTGCTGTCAAAAGCAACATACTGGTGGATGAACACACTTATTATATCTGCTCACAAA
AAGCCTATTGATCTGAAGGCAATTGGAAAATTGCCAATAGCAATGAGAGCAGTAACAAAT
TATGTTTGCCTGAAAGATGCATATGAAGAACAAAAGAAAAAAGTTGCAGATCATCCAAAT
CGGACTCCATCTATATGGCTTGCAATGTACAGAGCTTTTGGGCGACCAATTCTACTTAGT
AGCACATTCCGCTATCTGGCTGATTTACTGGGTTTTGCTGGACCTCTTTGTATTTCTGGA
ATAGTTCAGCGTGTGAATGAAACCCAGAATGGGACAAATAACACAACTGGAATTTCAGAA
ACCCTCTCATCAAAGGAATTTCTTGAAAACGCTTACGTTCTAGCAGTTCTTCTCTTCTTG
GCTCTTATTCTGCAAAGGACATTTTTGCAGGCTTCCTACTATGTAACCATAGAGACTGGC
ATTAACCTCCGTGGAGCTCTGCTGGCCATGATTTATAATAAAATCCTTAGGCTCTCTACG
TCTAACTTATCCATGGGGGAGATGACTCTGGGGCAGATCAACAACTTAGTCGCCATTGAA
ACTAATCAACTCATGTGGTTTTTGTTCCTGTGTCCCAATCTATGGGCTATGCCTGTTCAG
ATCATAATGGGCGTGATTCTGCTCTATAATTTACTTGGATCAAGTGCATTGGTCGGTGCA
GCTGTCATTGTGCTCCTTGCGCCAATTCAGTACTTTATTGCTACAAAGTTGGCAGAGGCT
CAGAAAAGTACACTTGATTATTCCACTGAGAGACTCAAGAAAACAAATGAAATATTGAAA
GGCATCAAACTTCTAAAATTGTATGCCTGGGAACACATTTTCTGCAAAAGTGTGGAGGAA
ACAAGAATGAAAGAACTATCTAGTCTCAAAACCTTTGCACTATATACATCACTCTCCATC
TTCATGAATGCAGCAATTCCCATAGCAGCTGTTCTTGCTACATTTGTGACCCATGCGTAT
GCCAGTGGAAACAATCTGAAACCTGCAGAGGCCTTTGCTTCACTGTCTCTCTTCCATATC
CTGGTCACACCACTGTTCCTGCTCTCCACGGTGGTCAGATTTGCAGTCAAAGCCATCATA
AGTGTTCAAAAGCTGAATGAGTTTCTCTTGAGTGATGAGATTGGTGACGACAGTTGGCGA
ACTGGTGAAAGTTCGCTTCCTTTTGAGTCCTGTAAGAAGCACACTGGAGTTCAGCCAAAA
ACTATAAACAGGAAACAGCCTGGAAGATATCACCTGGACAGCTATGAGCAATCAACACGG
CGTCTACGTCCCGCAGAAACAGAGGACATTGCAATAAAGGTCACAAATGGATACTTTTCA
TGGGGCAGTGGTTTAGCTACATTATCCAATATAGATATTCGAATTCCAACAGGTCAGTTA
ACCATGATTGTGGGCCAAGTAGGATGTGGGAAGTCCTCTCTTCTCCTTGCCATCCTCGGT
GAGATGCAGACATTGGAAGGAAAAGTTCACTGGAGCAATGTAAATGAATCTGAGCCTTCT
TTTGAAGCAACCAGAAGTAGGAACAGGTACTCTGTGGCATATGCAGCTCAAAAGCCTTGG
CTATTAAATGCTACAGTAGAAGAAAATATTACTTTTGGAAGTCCTTTTAACAAACAGAGG
TACAAAGCTGTCACAGATGCCTGTTCTCTTCAGCCAGATATTGACTTATTACCATTTGGA
GATCAAACTGAAATTGGAGAGAGGGGCATCAACCTGAGTGGGGGACAGAGGCAGAGAATC
TGTGTGGCACGAGCGCTGTATCAAAACACCAACATTGTCTTTTTGGATGATCCATTCTCA
GCCCTGGACATTCACTTGAGTGATCATTTAATGCAGGAGGGGATTTTGAAATTCCTGCAA
GATGACAAAAGGACACTCGTTCTTGTGACTCACAAATTACAGTATCTGACGCATGCTGAC
TGGATCATAGCCATGAAAGATGGAAGTGTCCTAAGAGAAGGAACTTTGAAGGACATTCAA
ACCAAAGATGTTGAGCTTTATGAACACTGGAAAACACTTATGAATCGGCAAGATCAAGAA
TTAGAAAAGGATATGGAAGCTGACCAAACTACTTTAGAGAGGAAAACTCTCCGACGGGCC
ATGTATTCAAGAGAAGCCAAAGCCCAGATGGAGGACGAAGACGAAGAGGAAGAAGAGGAG
GAAGATGAGGATGATAACATGTCCACTGTAATGAGGCTCAGGACTAAAATGCCATGGAAA
ACCTGCTGGCGCTACCTGACATCTGGAGGATTCTTCCTGCTCATCCTGATGATTTTCTCT
AAGCTTTTGAAGCATTCGGTCATTGTAGCTATAGACTATTGGCTGGCCACATGGACATCG
GAGTACAGTATAAACAATACTGGAAAAGCTGATCAGACCTACTATGTGGCTGGCTTTAGC
ATACTCTGTGGAGCAGGCATTTTCCTTTGCCTTGTTACATCCCTCACTGTAGAATGGATG
GGTCTCACAGCTGCCAAAAATCTTCACCACAACCTTCTCAATAAGATAATCCTTGGACCA
ATAAGGTTTTTTGATACCACACCCCTGGGACTGATTCTCAATCGCTTTTCAGCTGATACT
AATATCATTGATCAGCACATCCCTCCAACCTTGGAATCTCTAACTCGCTCAACACTGCTC
TGCCTGTCTGCCATTGGGATGATTTCTTATGCTACTCCTGTGTTCCTGGTTGCTCTCCTG
CCCCTTGGTGTTGCCTTTTATTTTATCCAGAAATACTTTCGGGTTGCCTCTAAGGACCTC
CAGGAACTTGACGATAGTACCCAGCTCCCTCTGCTCTGTCACTTCTCAGAAACAGCAGAA
GGACTCACCACCATTCGGGCCTTTAGGCATGAAACCAGATTTAAACAACGTATGCTGGAA
CTGACGGATACAAACAACATTGCCTACTTATTTCTCTCAGCTGCCAACAGATGGCTGGAG
GTCAGGACGGATTATCTGGGAGCTTGCATTGTCCTCACTGCATCTATAGCATCCATTAGT
GGGTCTTCGAATTCTGGATTGGTAGGCTTGGGTCTTCTGTATGCACTTACGATAACCAAT
TATTTGAATTGGGTTGTGAGGAACTTGGCTGACCTGGAGGTCCAGATGGGTGCAGTGAAG
AAGGTGAACAGTTTCCTGACTATGGAGTCAGAGAACTATGAAGGCACAATGGATCCTTCT
CAAGTTCCAGAACATTGGCCACAAGAAGGGGAGATCAAGATACATGATCTGTGTGTCAGA
TATGAAAATAATCTGAAACCTGTTCTTAAGCACGTCAAGGCTTACATCAAACCTGGACAA
AAGGTGGGCATATGTGGTCGCACTGGCAGTGGGAAATCATCGTTATCTCTGGCTTTCTTC
AGAATGGTTGATATATTTGATGGAAAAATTGTCATTGATGGGATAGACATTTCCAAATTA
CCACTGCACACACTACGTTCTAGACTTTCAATCATTCTGCAGGATCCAATACTATTCAGT
GGTTCCATTAGATTTAATTTAGATCCAGAGTGCAAATGCACAGATGACAGACTCTGGGAA
GCCTTAGAAATTGCTCAGCTGAAGAATATGGTCAAATCTCTACCTGGAGGTCTAGATGCG
GTTGTCACTGAAGGTGGGGAGAATTTTAGCGTTGGACAGAGACAGCTATTTTGCCTTGCC
AGGGCCTTTGTCCGCAAAAGCAGCATTCTTATTATGGATGAGGCAACAGCTTCCATTGAC
ATGGCCACAGAGAATATTTTGCAAAAAGTAGTAATGACAGCCTTTGCAGACCGGACCGTG
GTGACAATAGCTCACCGTGTCTCTTCTATTATGGATGCAGGCCTTGTTTTAGTCTTTTCT
GAGGGTATTTTAGTGGAGTGTGATACTGTCCCAAATTTGCTCGCCCACAAGAATGGCCTC
TTTTCCACTTTGGTGATGACCAACAAGTAG
GenBank Gene IDAF061323
GeneCard IDNot Available
GenAtlas IDABCC9
HGNC IDHGNC:60
Chromosome Location12
Locus12p12.1
References
  1. Aguilar-Bryan L, Clement JP 4th, Gonzalez G, Kunjilwar K, Babenko A, Bryan J: Toward understanding the assembly and structure of KATP channels. Physiol Rev. 1998 Jan;78(1):227-45. 9457174
  2. Scherer SE, Muzny DM, Buhay CJ, Chen R, Cree A, Ding Y, Dugan-Rocha S, Gill R, Gunaratne P, Harris RA, Hawes AC, Hernandez J, Hodgson AV, Hume J, Jackson A, Khan ZM, Kovar-Smith C, Lewis LR, Lozado RJ, Metzker ML, Milosavljevic A, Miner GR, Montgomery KT, Morgan MB, Nazareth LV, Scott G, Sodergren E, Song XZ, Steffen D, Lovering RC, Wheeler DA, Worley KC, Yuan Y, Zhang Z, Adams CQ, Ansari-Lari MA, Ayele M, Brown MJ, Chen G, Chen Z, Clerc-Blankenburg KP, Davis C, Delgado O, Dinh HH, Draper H, Gonzalez-Garay ML, Havlak P, Jackson LR, Jacob LS, Kelly SH, Li L, Li Z, Liu J, Liu W, Lu J, Maheshwari M, Nguyen BV, Okwuonu GO, Pasternak S, Perez LM, Plopper FJ, Santibanez J, Shen H, Tabor PE, Verduzco D, Waldron L, Wang Q, Williams GA, Zhang J, Zhou J, Allen CC, Amin AG, Anyalebechi V, Bailey M, Barbaria JA, Bimage KE, Bryant NP, Burch PE, Burkett CE, Burrell KL, Calderon E, Cardenas V, Carter K, Casias K, Cavazos I, Cavazos SR, Ceasar H, Chacko J, Chan SN, Chavez D, Christopoulos C, Chu J, Cockrell R, Cox CD, Dang M, Dathorne SR, David R, Davis CM, Davy-Carroll L, Deshazo DR, Donlin JE, D'Souza L, Eaves KA, Egan A, Emery-Cohen AJ, Escotto M, Flagg N, Forbes LD, Gabisi AM, Garza M, Hamilton C, Henderson N, Hernandez O, Hines S, Hogues ME, Huang M, Idlebird DG, Johnson R, Jolivet A, Jones S, Kagan R, King LM, Leal B, Lebow H, Lee S, LeVan JM, Lewis LC, London P, Lorensuhewa LM, Loulseged H, Lovett DA, Lucier A, Lucier RL, Ma J, Madu RC, Mapua P, Martindale AD, Martinez E, Massey E, Mawhiney S, Meador MG, Mendez S, Mercado C, Mercado IC, Merritt CE, Miner ZL, Minja E, Mitchell T, Mohabbat F, Mohabbat K, Montgomery B, Moore N, Morris S, Munidasa M, Ngo RN, Nguyen NB, Nickerson E, Nwaokelemeh OO, Nwokenkwo S, Obregon M, Oguh M, Oragunye N, Oviedo RJ, Parish BJ, Parker DN, Parrish J, Parks KL, Paul HA, Payton BA, Perez A, Perrin W, Pickens A, Primus EL, Pu LL, Puazo M, Quiles MM, Quiroz JB, Rabata D, Reeves K, Ruiz SJ, Shao H, Sisson I, Sonaike T, Sorelle RP, Sutton AE, Svatek AF, Svetz LA, Tamerisa KS, Taylor TR, Teague B, Thomas N, Thorn RD, Trejos ZY, Trevino BK, Ukegbu ON, Urban JB, Vasquez LI, Vera VA, Villasana DM, Wang L, Ward-Moore S, Warren JT, Wei X, White F, Williamson AL, Wleczyk R, Wooden HS, Wooden SH, Yen J, Yoon L, Yoon V, Zorrilla SE, Nelson D, Kucherlapati R, Weinstock G, Gibbs RA: The finished DNA sequence of human chromosome 12. Nature. 2006 Mar 16;440(7082):346-51. 16541075
  3. Babenko AP, Gonzalez G, Aguilar-Bryan L, Bryan J: Reconstituted human cardiac KATP channels: functional identity with the native channels from the sarcolemma of human ventricular cells. Circ Res. 1998 Nov 30;83(11):1132-43. 9831708
  4. Allebrandt KV, Amin N, Muller-Myhsok B, Esko T, Teder-Laving M, Azevedo RV, Hayward C, van Mill J, Vogelzangs N, Green EW, Melville SA, Lichtner P, Wichmann HE, Oostra BA, Janssens AC, Campbell H, Wilson JF, Hicks AA, Pramstaller PP, Dogas Z, Rudan I, Merrow M, Penninx B, Kyriacou CP, Metspalu A, van Duijn CM, Meitinger T, Roenneberg T: A K(ATP) channel gene effect on sleep duration: from genome-wide association studies to function in Drosophila. Mol Psychiatry. 2013 Jan;18(1):122-32. doi: 10.1038/mp.2011.142. Epub 2011 Nov 22. 22105623
  5. Bienengraeber M, Olson TM, Selivanov VA, Kathmann EC, O'Cochlain F, Gao F, Karger AB, Ballew JD, Hodgson DM, Zingman LV, Pang YP, Alekseev AE, Terzic A: ABCC9 mutations identified in human dilated cardiomyopathy disrupt catalytic KATP channel gating. Nat Genet. 2004 Apr;36(4):382-7. Epub 2004 Mar 21. 15034580
  6. Olson TM, Alekseev AE, Moreau C, Liu XK, Zingman LV, Miki T, Seino S, Asirvatham SJ, Jahangir A, Terzic A: KATP channel mutation confers risk for vein of Marshall adrenergic atrial fibrillation. Nat Clin Pract Cardiovasc Med. 2007 Feb;4(2):110-6. 17245405
  7. van Bon BW, Gilissen C, Grange DK, Hennekam RC, Kayserili H, Engels H, Reutter H, Ostergaard JR, Morava E, Tsiakas K, Isidor B, Le Merrer M, Eser M, Wieskamp N, de Vries P, Steehouwer M, Veltman JA, Robertson SP, Brunner HG, de Vries BB, Hoischen A: Cantu syndrome is caused by mutations in ABCC9. Am J Hum Genet. 2012 Jun 8;90(6):1094-101. doi: 10.1016/j.ajhg.2012.04.014. Epub 2012 May 17. 22608503
  8. Harakalova M, van Harssel JJ, Terhal PA, van Lieshout S, Duran K, Renkens I, Amor DJ, Wilson LC, Kirk EP, Turner CL, Shears D, Garcia-Minaur S, Lees MM, Ross A, Venselaar H, Vriend G, Takanari H, Rook MB, van der Heyden MA, Asselbergs FW, Breur HM, Swinkels ME, Scurr IJ, Smithson SF, Knoers NV, van der Smagt JJ, Nijman IJ, Kloosterman WP, van Haelst MM, van Haaften G, Cuppen E: Dominant missense mutations in ABCC9 cause Cantu syndrome. Nat Genet. 2012 May 18;44(7):793-6. doi: 10.1038/ng.2324. 22610116