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3.A.1.208.23
The Sur2B (ABCC9) sulfonylurea receptor. The amino-terminal transmembrane domain of Sur2B binds Kir6.2 (Winkler et al., 2011). Dominant missense mutations in ABCC9, promoting open channel formation, cause Cantú syndrome (Harakalova et al., 2012; van Bon et al., 2012). This protein is part of an ATP-dependent potassium (K(ATP)) channel that couples the metabolic state of a cell with its electrical activity.  Associated with early repolarization (ERS) and Brugada (BrS) syndromes (Hu et al. 2014).  This ATP-sensitive potassium (K(ATP)) channel couples glucose metabolism to insulin secretion in pancreatic beta-cells (de Wet et al. 2007).

Accession Number:O60706
Protein Name:ATP-binding cassette sub-family C member 9
Length:1549
Molecular Weight:174223.00
Species:Homo sapiens (Human) [9606]
Number of TMSs:19
Location1 / Topology2 / Orientation3: Membrane1 / Multi-pass membrane protein2
Substrate Sulfonylurea

Cross database links:

Genevestigator: O60706
eggNOG: prNOG18296
Entrez Gene ID: 10060   
Pfam: PF00664    PF00005   
KEGG: hsa:10060   

Gene Ontology

GO:0008282 C:ATP-sensitive potassium channel complex
GO:0005524 F:ATP binding
GO:0042626 F:ATPase activity, coupled to transmembrane movement of substances
GO:0015459 F:potassium channel regulator activity
GO:0008281 F:sulfonylurea receptor activity
GO:0051607 P:defense response to virus
GO:0010107 P:potassium ion import

References (4)

[1] “Toward understanding the assembly and structure of KATP channels.”  Aguilar-Bryan L.et.al.   9457174
[2] “The finished DNA sequence of human chromosome 12.”  Scherer S.E.et.al.   16541075
[3] “ABCC9 mutations identified in human dilated cardiomyopathy disrupt catalytic KATP channel gating.”  Bienengraeber M.et.al.   15034580
[4] “KATP channel mutation confers risk for vein of Marshall adrenergic atrial fibrillation.”  Olson T.M.et.al.   17245405

External Searches:

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  • Search COGs (Clusters of Orthologous Groups of proteins)
  • 2° Structure (Network Protein Sequence Analysis)

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Predict TMSs (Predict number of transmembrane segments)
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FASTA formatted sequence
1:	MSLSFCGNNI SSYNINDGVL QNSCFVDALN LVPHVFLLFI TFPILFIGWG SQSSKVQIHH 
61:	NTWLHFPGHN LRWILTFALL FVHVCEIAEG IVSDSRRESR HLHLFMPAVM GFVATTTSIV 
121:	YYHNIETSNF PKLLLALFLY WVMAFITKTI KLVKYCQSGL DISNLRFCIT GMMVILNGLL 
181:	MAVEINVIRV RRYVFFMNPQ KVKPPEDLQD LGVRFLQPFV NLLSKATYWW MNTLIISAHK 
241:	KPIDLKAIGK LPIAMRAVTN YVCLKDAYEE QKKKVADHPN RTPSIWLAMY RAFGRPILLS 
301:	STFRYLADLL GFAGPLCISG IVQRVNETQN GTNNTTGISE TLSSKEFLEN AYVLAVLLFL 
361:	ALILQRTFLQ ASYYVTIETG INLRGALLAM IYNKILRLST SNLSMGEMTL GQINNLVAIE 
421:	TNQLMWFLFL CPNLWAMPVQ IIMGVILLYN LLGSSALVGA AVIVLLAPIQ YFIATKLAEA 
481:	QKSTLDYSTE RLKKTNEILK GIKLLKLYAW EHIFCKSVEE TRMKELSSLK TFALYTSLSI 
541:	FMNAAIPIAA VLATFVTHAY ASGNNLKPAE AFASLSLFHI LVTPLFLLST VVRFAVKAII 
601:	SVQKLNEFLL SDEIGDDSWR TGESSLPFES CKKHTGVQPK TINRKQPGRY HLDSYEQSTR 
661:	RLRPAETEDI AIKVTNGYFS WGSGLATLSN IDIRIPTGQL TMIVGQVGCG KSSLLLAILG 
721:	EMQTLEGKVH WSNVNESEPS FEATRSRNRY SVAYAAQKPW LLNATVEENI TFGSPFNKQR 
781:	YKAVTDACSL QPDIDLLPFG DQTEIGERGI NLSGGQRQRI CVARALYQNT NIVFLDDPFS 
841:	ALDIHLSDHL MQEGILKFLQ DDKRTLVLVT HKLQYLTHAD WIIAMKDGSV LREGTLKDIQ 
901:	TKDVELYEHW KTLMNRQDQE LEKDMEADQT TLERKTLRRA MYSREAKAQM EDEDEEEEEE 
961:	EDEDDNMSTV MRLRTKMPWK TCWRYLTSGG FFLLILMIFS KLLKHSVIVA IDYWLATWTS 
1021:	EYSINNTGKA DQTYYVAGFS ILCGAGIFLC LVTSLTVEWM GLTAAKNLHH NLLNKIILGP 
1081:	IRFFDTTPLG LILNRFSADT NIIDQHIPPT LESLTRSTLL CLSAIGMISY ATPVFLVALL 
1141:	PLGVAFYFIQ KYFRVASKDL QELDDSTQLP LLCHFSETAE GLTTIRAFRH ETRFKQRMLE 
1201:	LTDTNNIAYL FLSAANRWLE VRTDYLGACI VLTASIASIS GSSNSGLVGL GLLYALTITN 
1261:	YLNWVVRNLA DLEVQMGAVK KVNSFLTMES ENYEGTMDPS QVPEHWPQEG EIKIHDLCVR 
1321:	YENNLKPVLK HVKAYIKPGQ KVGICGRTGS GKSSLSLAFF RMVDIFDGKI VIDGIDISKL 
1381:	PLHTLRSRLS IILQDPILFS GSIRFNLDPE CKCTDDRLWE ALEIAQLKNM VKSLPGGLDA 
1441:	VVTEGGENFS VGQRQLFCLA RAFVRKSSIL IMDEATASID MATENILQKV VMTAFADRTV 
1501:	VTIAHRVSSI MDAGLVLVFS EGILVECDTV PNLLAHKNGL FSTLVMTNK