Conserved Protein Domain Family
CFTR_protein

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TIGR01271: CFTR_protein 
cystic fibrosis transmembrane conductor regulator (CFTR)
The model describes the cystis fibrosis transmembrane conductor regulator (CFTR) in eukaryotes. The principal role of this protein is chloride ion conductance. The protein is predicted to consist of 12 transmembrane domains. Mutations or lesions in the genetic loci have been linked to the aetiology of asthma, bronchiectasis, chronic obstructive pulmonary disease etc. Disease-causing mutations have been studied by 36Cl efflux assays in vitro cell cultures and electrophysiology, all of which point to the impairment of chloride channel stability and not the biosynthetic processing per se. [Transport and binding proteins, Anions]
Statistics
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PSSM-Id: 273530
View PSSM: TIGR01271
Aligned: 3 rows
Threshold Bit Score: 2853.78
Threshold Setting Gi: 5052017
Created: 8-Oct-2014
Updated: 11-Oct-2014
Structure
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Aligned Rows:
Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
P26362      1 MQRSPIEKANAFSKLFFRWPRPILKKGYRQKLELSDIYQIPSSDSADELSEMLEREWDRELATsKKNPKLVNALRRCFFW 80   spiny dogfish
P13569      1 MQRSPLEKASVVSKLFFSWTRPILRKGYRQRLELSDIYQIPSVDSADNLSEKLEREWDRELAS-KKNPKLINALRRCFFW 79   human
AAD38404    1 MQKSPVEDANFLSKYFFWWTSPLLRKGFRKKLDLTDVYKAPSFDLADNLSERLEREWDREVASaKKKPRLLRALSRCFLW 80   Atlantic salmon
P26362     81 RFLFYGILLYFVEFTKAVQPLCLGRIIASYNAKNTYEREIAYYLALGLCLLFVVRTLFLHPAVFGLQHLGMQMRIALFSL 160  spiny dogfish
P13569     80 RFMFYGIFLYLGEVTKAVQPLLLGRIIASYDPDNKEERSIAIYLGIGLCLLFIVRTLLLHPAIFGLHHIGMQMRIAMFSL 159  human
AAD38404   81 PFVFYGILLYFGEASKTVQPQLLGRIIGSFDPFHAPEREQGYYLALGLSLLFIARFLLLQPAIFGLHHLGMQIRIALFSL 160  Atlantic salmon
P26362    161 IYKKILKMSSRVLDKIDTGQLVSLLSNNLNKFDEGVAVAHFVWIAPVQVVLLMGLIWNELTEFVFCGLGFLIMLALFQAW 240  spiny dogfish
P13569    160 IYKKTLKLSSRVLDKISIGQLVSLLSNNLNKFDEGLALAHFVWIAPLQVALLMGLIWELLQASAFCGLGFLIVLALFQAG 239  human
AAD38404  161 IYKKTLKLSSRVLDKISTGQLVSLMSTHLNKLDESLGLAHFVWITPLQCILCVGLIWELIEVNGFCALAALTLLGIVQAC 240  Atlantic salmon
P26362    241 LGKKMMQYRDKRAGKINERLAITSEIIDNIQSVKVYCWEDAMEKIIDDIRQVELKLTRKVAYCRYFSSSAFFFSGFFVVF 320  spiny dogfish
P13569    240 LGRMMMKYRDQRAGKISERLVITSEMIENIQSVKAYCWEEAMEKMIENLRQTELKLTRKAAYVRYFNSSAFFFSGFFVVF 319  human
AAD38404  241 LSQKMGPHRQKRAGMISRRLALTSEIVENIHSVKAYGWEEVMETIIKNIRQDEMSLTRKIGSLRYFYSASYFFSAILVIV 320  Atlantic salmon
P26362    321 LSVVPYAFIHTIKLRRIFTTISYNIVLRMTVTRQFPSAIQTWYDSLGAIRKIQDFLHKDEHKTVEYNLTTKEVEMVNVTA 400  spiny dogfish
P13569    320 LSVLPYALIKGIILRKIFTTISFCIVLRMAVTRQFPWAVQTWYDSLGAINKIQDFLQKQEYKTLEYNLTTTEVVMENVTA 399  human
AAD38404  321 SAIVPHALSKGIILRRIFTTASYCMVLRMTLTRQLPGSIQMWYDTLALVTKIEEYLCKEEYKVLEYNLTTTEIELVNVTA 400  Atlantic salmon
P26362    401 SWDEGIGELFEKVKQNDSERKMANGDDGLFFSNFSLHVTPVLKNISFKLEKGELLAIAGSTGSGKSSLLMMIMGELEPSD 480  spiny dogfish
P13569    400 FWEEGFGELFEKAKQNNNNRKTSNGDDSLFFSNFSLLGTPVLKDINFKIERGQLLAVAGSTGAGKTSLLMVIMGELEPSE 479  human
AAD38404  401 SWDEGIGELFEKIKQENKANGQPNGDAGLFFTNL--YVTPVLKNISLYLEKGQMLAVAGSTGSGKSSLLMMILGELVPSE 478  Atlantic salmon
P26362    481 GKIKHSGRISYSPQVPWIMPGTIKDNIIFGLSYDEYRYTSVVNACQLEEDITVFPNKDKTVLGDGGITLSGGQRARISLA 560  spiny dogfish
P13569    480 GKIKHSGRISFCSQFSWIMPGTIKENIIFGVSYDEYRYRSVIKACQLEEDISKFAEKDNIVLGEGGITLSGGQRARISLA 559  human
AAD38404  479 GKIRHSGRISFSPQTSWIMPGTIRDNILFGLTYDEFRYTSVIKACQLEEDLALLPEKDKTPLMEGGVTLSGGQRARIGLA 558  Atlantic salmon
P26362    561 RALYKDADLYLLDSPFSHLDVTTEKDIFESCLCKLMVNKTRILVTSKLEHLKKADKILLLHEGHCYFYGTFSELQGEKPD 640  spiny dogfish
P13569    560 RAVYKDADLYLLDSPFGYLDVLTEKEIFESCVCKLMANKTRILVTSKMEHLKKADKILILHEGSSYFYGTFSELQNLQPD 639  human
AAD38404  559 RAVYKDADLYLLDSPFTHLDIVTEKEIFERCLCKLLSSKTRIVVTSKLEHLKRADKILLLHNGVCYFYGSFSELQAKRPD 638  Atlantic salmon
P26362    641 FSSQLLGSVHFDSFSAERRNSILTETFRRCSVSSgDGAglGSYSETRKASFKQPPp--------------------EFNE 700  spiny dogfish
P13569    640 FSSKLMGCDSFDQFSAERRNSILTETLHRFSLEG-DAP--VSWTETKKQSFKQTG---------------------EFGE 695  human
AAD38404  639 FSSLLLGLEAYDNIKAERRSSILTETLRRVSID--ESTv-FRGPEPIHQSFRQPPppiictnngpqaitiaggdglTAAE 715  Atlantic salmon
P26362    701 KRKSSlIVNPITSNKKFSLVQTAMSYPQT-----NGMEdaTSEPGERHFSLIPENELGEPTKPRSNIFKSELPFQ-AHRR 774  spiny dogfish
P13569    696 KRKNS-ILNPINSIRKFSIVQKTPLQMNG-----IEED--SDEPLERRLSLVPDSEQGEAILPRISVISTGPTLQ-ARRR 766  human
AAD38404  716 KRKQSiILNPLAPARKFSFIGMGPQKAQApppgsTTIEdaVRELSDRKFSVVPEDDQVEESLPRGNQYHHGLQHLsGQRR 795  Atlantic salmon
P26362    775 QSVLALMTHS-STSPNKIHARRSAVRKMSMLSQTNfaSSEIDIYSRRLSEDGSFEISEEINEEDLKECFADEEEIQNVTT 853  spiny dogfish
P13569    767 QSVLNLMTHSvNQGQNIHRKTTASTRKVSLAPQAN--LTELDIYSRRLSQETGLEISEEINEEDLKECFFDDMESIPAVT 844  human
AAD38404  796 QSVLQFITNA--RGEGRREQLQTSFRKKLSITPQCelASELDIYARPLSKDSVYDISEDVDEQDMEQCFADERENVFETT 873  Atlantic salmon
P26362    854 TWSTYLRYVTTNRNLVFVLILCLVIFLAEVAASLAGLWIISG-------LAintGSQTNDTSTDLShlSVFSKFITNGSH 926  spiny dogfish
P13569    845 TWNTYLRYITVHKSLIFVLIWCLVIFLAEVAASLVVLWLLGN-------TP---LQDKGNSTHSRN--NSYAVIITSTSS 912  human
AAD38404  874 SWNTYLRYIFTNRNLVYVLIFIFLVFAVEVAGSVLGIFLITDkiwteeaNPsapNYVDQQHANASSpdVQKPVIVTPTSA 953  Atlantic salmon
P26362    927 YYIFYIYVGLADSFLALGVIRGLPLVHTLVTVSKDLHKQMLHSVLQGPMTAFNKMKAGRILNRFIKDTAIIDDMLPLTVF 1006 spiny dogfish
P13569    913 YYVFYIYVGVADTLLAMGFFRGLPLVHTLITVSKILHHKMLHSVLQAPMSTLNTLKAGGILNRFSKDIAILDDLLPLTIF 992  human
AAD38404  954 YYIIYIYVATSESVLALGFFRGLPLVHTLLTVSKRLHEQMLTAVLRAPMAVLNTMKTGRIMNRFTKDMATIDDMLPLALF 1033 Atlantic salmon
P26362   1007 DFVQLILIVVGAICVVSVLQPYTLLAAIPVAVIFIMLRAYFLRTSQQLKQLESEARSPIFSHLITSLRGLWTVRAFGRQS 1086 spiny dogfish
P13569    993 DFIQLLLIVIGAIAVVAVLQPYIFVATVPVIVAFIMLRAYFLQTSQQLKQLESEGRSPIFTHLVTSLKGLWTLRAFGRQP 1072 human
AAD38404 1034 DLIQLTLIVLGAIFTVSIMRPYIFIAAIPLAVIFVVLRMYFLRTGQQLKQLEAEARSPIFSHLIISLKGLWTIRAFGRQS 1113 Atlantic salmon
P26362   1087 YFETLFHKALNLHTANWFLYLSTLRWFQMRIDIVFVLFFIAVTFIAIATHDVGEGQVGIILTLAMNITSTLQWAVNSSID 1166 spiny dogfish
P13569   1073 YFETLFHKALNLHTANWFLYLSTLRWFQMRIEMIFVIFFIAVTFISILTTGEGEGRVGIILTLAMNIMSTLQWAVNSSID 1152 human
AAD38404 1114 YFETLFHKALNTHTAIWFHYLATLRWFLFRCDIIFVFFFSAAVFIAVGTNQDKPGEIGIIVALAMLILGTFQWAVITSIT 1193 Atlantic salmon
P26362   1167 VDGLMRSVSRVFKYIDIPPEgSETKNRHNAN---NPSDVLVIENKHLTK--EWPSGGQMMVNNLTAKYTSDGRAVLQDLS 1241 spiny dogfish
P13569   1153 VDSLMRSVSRVFKFIDMPTE-GKPTKSTKPYkngQLSKVMIIENSHVKKddIWPSGGQMTVKDLTAKYTEGGNAILENIS 1231 human
AAD38404 1194 VDGLMRSVDRVFKFIDLPQEePRPSGGGGK------GTDLVIDNPHAQD--CWPNQGHMDVQGLTVKYTEAGRSVLQDLS 1265 Atlantic salmon
P26362   1242 FSVNAGQRVGLLGRTGAGKSTLLSALLRLLSTEGEIQIDGISWNSVSLQKWRKAFGVIPQKVFVFSGTFRKNLDPYEQWS 1321 spiny dogfish
P13569   1232 FSISPGQRVGLLGRTGSGKSTLLSAFLRLLNTEGEIQIDGVSWDSITLQQWRKAFGVIPQKVFIFSGTFRKNLDPYEQWS 1311 human
AAD38404 1266 FTVEGGQSVGVLGRTGSGKSTLLSALLRLASTDGEISIDGVSWNSVTLQTWRKAFGVVPQKIFILTGTFRMNLDPHARYS 1345 Atlantic salmon
P26362   1322 DEEIWKVTEEVGLKSMIEQFPDKLNFVLVDGGYILSNGHKQLMCLARSILSKAKILLLDEPTAHLDPVTFQIIRKTLKHT 1401 spiny dogfish
P13569   1312 DQEIWKVADEVGLRSVIEQFPGKLDFVLVDGGCVLSHGHKQLMCLARSVLSKAKILLLDEPSAHLDPVTYQIIRRTLKQA 1391 human
AAD38404 1346 DEELWRVAEEVGLKSVIEQFPDKLDFQLEDGGYVLSNGHKQLMCLARSILSKARILLLDEPSAFLDPITLQVLRKTLKQS 1425 Atlantic salmon
P26362   1402 FSNCTVILSEHRVEALLECQQFLVIEGCSVKQFDALQKLLTEASLFKQVFGHLDRAKLFTahRRNSSKRKTR---PKISA 1478 spiny dogfish
P13569   1392 FADCTVILCEHRIEAMLECQQFLVIEENKVRQYDSIQKLLNERSLFRQAISPSDRVKLFP--HRNSSKCKSK---PQIAA 1466 human
AAD38404 1426 FSNCTVILSEHRVEPLLECQSFLMIEGSSMKRYDSIQKLLNETSHLKQAMSAAERLKLFPlhRLNSSKRPPQsqqPKITA 1505 Atlantic salmon
P26362   1479 LQEEAEEDLQETRL 1492 spiny dogfish
P13569   1467 LKEETEEEVQDTRL 1480 human
AAD38404 1506 LREVAEDEVQNTHL 1519 Atlantic salmon
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