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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). The vasodilator binding sites on the SUR2 subunit have been identified (Ding et al. 2022). Cryo-EM structures of SUR2A and SUR2B subunits in complex with Mg-nucleotides and P1075 or levcromakalim, two chemically distinct KATP openers that are specific to SUR2, have been determined. Both P1075 and levcromakalim bind to a common site in the transmembrane domain (TMD) of the SUR2 subunit, which is between TMD1 and TMD2 and is embraced by TMSs 10, 11, 12, 14, and 17. These KATP openers synergize with Mg-nucleotides to stabilize SUR2 in the NBD-dimerized occluded state to activate the channel (Ding et al. 2022).

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

Cross database links:

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

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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