Gene name: PKHD1

Uniprot entry:

P08F94

Protein names:

Fibrocystin (Polycystic kidney and hepatic disease 1 protein) (Polyductin) (Tigmin)

Protein sequence:

1_MTAWL 6_ ISLMS 11_ IEVLL 16_ LAVRH 21_ LSLHI 26_ EPEEG 31_ SLAGG 36_ TWITV 41_ IFDGL 46_ ELGVL 51_ YPNNG 56_ SQLEI 61_ HLVNV 66_ NMVVP 71_ ALRSV 76_ PCDVF 81_ PVFLD 86_ LPVVT 91_ CRTRS 96_ VLSEA 101_ HEGLY 106_ FLEAY 111_ FGGQL 116_ VSSPN 121_ PGPRD 126_ SCTFK 131_ FSKAQ 136_ TPIVH 141_ QVYPP 146_ SGVPG 151_ KLIHV 156_ YGWII 161_ TGRLE 166_ TFDFD 171_ AEYID 176_ SPVIL 181_ EAQGD 186_ KWVTP 191_ CSLIN 196_ RQMGS 201_ CYPIQ 206_ EDHGL 211_ GTLQC 216_ HVEGD 221_ YIGSQ 226_ NVSFS 231_ VFNKG 236_ KSMVH 241_ KKAWL 246_ ISAKQ 251_ DLFLY 256_ QTHSE 261_ ILSVF 266_ PETGS 271_ LGGRT 276_ NITIT 281_ GDFFD 286_ NSAQV 291_ TIAGI 296_ PCDIR 301_ HVSPR 306_ KIECT 311_ TRAPG 316_ KDVRL 321_ TTPQP 326_ GNRGL 331_ LFEVG 336_ DAVEG 341_ LELTE 346_ ATPGY 351_ RWQIV 356_ PNASS 361_ PFGFW 366_ SQEGQ 371_ PFRAR 376_ LSGFF 381_ VAPET 386_ NNYTF 391_ WIQAD 396_ SQASL 401_ HFSWS 406_ EEPRT 411_ KVKVA 416_ SISVG 421_ TADWF 426_ DSWEQ 431_ NRDEG 436_ TWQQK 441_ TPKLE 446_ LLGGA 451_ MYYLE 456_ AEHHG 461_ IAPSR 466_ GMRIG 471_ VQIHN 476_ TWLNP 481_ DVVTT 486_ YLREK 491_ HQIRV 496_ RAQRL 501_ PEVQV 506_ LNVSG 511_ RGNFF 516_ LTWDN 521_ VSSQP 526_ IPANA 531_ TAHLI 536_ QTTIE 541_ ELLAV 546_ KCKLE 551_ PLWSN 556_ ILLRL 561_ GFERG 566_ PEVSN 571_ SDGDL 576_ TSGTE 581_ PFCGR 586_ FSLRQ 591_ PRHLV 596_ LTPPA 601_ AQKGY 606_ RLDQY 611_ THLCL 616_ AYKGH 621_ MNKIL 626_ KMIVS 631_ FTIGF 636_ QNMVK 641_ NTTCD 646_ WSLTR 651_ TSPES 656_ WQFDC 661_ TDLWE 666_ TCVRC 671_ FGDLQ 676_ PPPAN 681_ SPVLV 686_ HQINL 691_ LPLAQ 696_ ETGLF 701_ YVDEI 706_ IIADT 711_ NVTVS 716_ QADSG 721_ TARPG 726_ GNLVE 731_ SVSVV 736_ GSPPV 741_ YSVTS 746_ WLAGC 751_ GTELP 756_ LITAR 761_ SVPTE 766_ GTEEG 771_ SGLVL 776_ VTTQR 781_ RQRTS 786_ PPLGG 791_ HFRIQ 796_ LPNTV 801_ ISDVP 806_ VQISA 811_ HHLHQ 816_ LLQNN 821_ ADDFT 826_ SRYLN 831_ ASDFT 836_ VKEDL 841_ YTCYE 846_ HVWTL 851_ SWSTQ 856_ IGDLP 861_ NFIRV 866_ SDENL 871_ TGVNP 876_ AAATR 881_ VVYDG 886_ GVFLG 891_ PIFGD 896_ MLATA 901_ NQHTQ 906_ VVVRV 911_ NDVPA 916_ HCPGS 921_ CSFQY 926_ LQGST 931_ PCVHS 936_ VWYSI 941_ DGDIN 946_ LMIYI 951_ TGTGF 956_ SGDSQ 961_ FLQVT 966_ VNKTS 971_ CKVIF 976_ SNQTN 981_ VVCQT 986_ DLLPV 991_ GMHRI 996_ LMLVR 1001_ PSGLA 1006_ ISATG 1011_ EDLFL 1016_ NVKPR 1021_ LDMVE 1026_ PSRAA 1031_ DIGGL 1036_ WATIR 1041_ GSSLE 1046_ GVSLI 1051_ LFGSY 1056_ SCAIN 1061_ VATSN 1066_ SSRIQ 1071_ CKVPP 1076_ RGKDG 1081_ RIVNV 1086_ TVIRG 1091_ DYSAV 1096_ LPRAF 1101_ TYVSS 1106_ LNPVI 1111_ VTLSR 1116_ NISNI 1121_ AGGET 1126_ LVIGV 1131_ ARLMN 1136_ YTDLD 1141_ VEVHV 1146_ QDALA 1151_ PVHTQ 1156_ SAWGL 1161_ EVALP 1166_ PLPAG 1171_ LHRIS 1176_ VSING 1181_ VSIHS 1186_ QGVDL 1191_ HIQYL 1196_ TEVFS 1201_ IEPCC 1206_ GSLLG 1211_ GTILS 1216_ ISGIG 1221_ FSRDP 1226_ ALVWV 1231_ LVGNR 1236_ SCDIV 1241_ NLTEA 1246_ SIWCE 1251_ TLPAP 1256_ QIPDA 1261_ GAPTV 1266_ PAAVE 1271_ VWAGN 1276_ RFFAR 1281_ GPSPS 1286_ LVGKG 1291_ FTFMY 1296_ EAAAT 1301_ PVVTA 1306_ MQGEI 1311_ TNSSL 1316_ SLHVG 1321_ GSNLS 1326_ NSVIL 1331_ LGNLN 1336_ CDVET 1341_ QSFQG 1346_ NVSLS 1351_ GCSIP 1356_ LHSLE 1361_ AGIYP 1366_ LQVRQ 1371_ KQMGF 1376_ ANMSV 1381_ VLQQF 1386_ AVMPR 1391_ IMAIF 1396_ PSQGS 1401_ ACGGT 1406_ ILTVR 1411_ GLLLN 1416_ SRRRS 1421_ VRVDL 1426_ SGPFT 1431_ CVILS 1436_ LGDHT 1441_ ILCQV 1446_ SLEGD 1451_ PLPGA 1456_ SFSLN 1461_ VTVLV 1466_ NGLTS 1471_ ECQGN 1476_ CTLFI 1481_ REEAS 1486_ PVMDA 1491_ LSTNT 1496_ SGSLT 1501_ TVLIR 1506_ GQRLA 1511_ TTADE 1516_ PMVFV 1521_ DDQLP 1526_ CNVTF 1531_ FNASH 1536_ VVCQT 1541_ RDLAP 1546_ GPHYL 1551_ SVFYT 1556_ RNGYA 1561_ CSGNV 1566_ SRHFY 1571_ IMPQV 1576_ FHYFP 1581_ KNFSL 1586_ HGGSL 1591_ LTIEG 1596_ TGLRG 1601_ QNTTS 1606_ VYIDQ 1611_ QTCLT 1616_ VNIGA 1621_ ELIRC 1626_ IVPTG 1631_ NGSVA 1636_ LEIEV 1641_ DGLWY 1646_ HIGVI 1651_ GYNKA 1656_ FTPEL 1661_ ISISQ 1666_ SDDIL 1671_ TFAVA 1676_ QISGA 1681_ ANIDI 1686_ FIGMS 1691_ PCVGV 1696_ SGNHT 1701_ VLQCV 1706_ VPSLP 1711_ AGEYH 1716_ VRGYD 1721_ CIRGW 1726_ ASSAL 1731_ VFTSR 1736_ VIITA 1741_ VTENF 1746_ GCLGG 1751_ RLVHV 1756_ FGAGF 1761_ SPGNV 1766_ SAAVC 1771_ GAPCR 1776_ VLANA 1781_ TVSAF 1786_ SCLVL 1791_ PLDVS 1796_ LAFLC 1801_ GLKRE 1806_ EDSCE 1811_ AARHT 1816_ YVQCD 1821_ LTVAM 1826_ ATEQL 1831_ LESWP 1836_ YLYIC 1841_ EESSQ 1846_ CLFVP 1851_ DHWAE 1856_ SMFPS 1861_ FSGLF 1866_ ISPKL 1871_ ERDEV 1876_ LIYNS 1881_ SCNIT 1886_ METEA 1891_ EMECE 1896_ TPNQP 1901_ ITVKI 1906_ TEIRK 1911_ RWGQN 1916_ TQGNF 1921_ SLQFC 1926_ RRWSR 1931_ THSWF 1936_ PERLP 1941_ QDGDN 1946_ VTVEN 1951_ GQLLL 1956_ LDTNT 1961_ SILNL 1966_ LHIKG 1971_ GKLIF 1976_ MAPGP 1981_ IELRA 1986_ HAILV 1991_ SDGGE 1996_ LRIGS 2001_ EDKPF 2006_ QGRAQ 2011_ ITLYG 2016_ SSYST 2021_ PFFPY 2026_ GVKFL 2031_ AVRNG 2036_ TLSLH 2041_ GSLPE 2046_ VIVTC 2051_ LRATA 2056_ HALDT 2061_ VLALE 2066_ DAVDW 2071_ NPGDE 2076_ VVIIS 2081_ GTGVK 2086_ GAKPM 2091_ EEIVT 2096_ VETVQ 2101_ DTDLY 2106_ LKSPL 2111_ RYSHN 2116_ FTENW 2121_ VAGEH 2126_ HILKA 2131_ TVALL 2136_ SRSIT 2141_ IQGNL 2146_ TNERE 2151_ KLLVS 2156_ CQEAN 2161_ APEGN 2166_ LQHCL 2171_ YSMSE 2176_ KMLGS 2181_ RDMGA 2186_ RVIVQ 2191_ SFPEE 2196_ PSQVQ 2201_ LKGVQ 2206_ FQVLG 2211_ QAFHK 2216_ HLSSL 2221_ TLVGA 2226_ MRESF 2231_ IQGCT 2236_ VRNSF 2241_ SRGLS 2246_ MCGTL 2251_ GLKVD 2256_ SNVFY 2261_ NILGH 2266_ ALLVG 2271_ TCTEM 2276_ RYISW 2281_ EAIHG 2286_ RKDDW 2291_ SGHGN 2296_ IIRNN 2301_ VIIQV 2306_ SGAEG 2311_ LSNPE 2316_ MLTPS 2321_ GIYIC 2326_ SPTNV 2331_ IEGNR 2336_ VCGAG 2341_ YGYFF 2346_ HLMTN 2351_ QTSQA 2356_ PLLSF 2361_ TQNIA 2366_ HSCTR 2371_ YGLFV 2376_ YPKFQ 2381_ PPWDN 2386_ VTGTT 2391_ LFQSF 2396_ TVWES 2401_ AGGAQ 2406_ IFRSS 2411_ NLRLK 2416_ NFKVY 2421_ SCRDF 2426_ GIDVL 2431_ ESDAN 2436_ TSVTD 2441_ SLLLG 2446_ HFAHK 2451_ GSLCM 2456_ SSGIK 2461_ TPKRW 2466_ ELMVS 2471_ NTTFV 2476_ NFDLI 2481_ NCVAI 2486_ RTCSD 2491_ CSQGQ 2496_ GGFTV 2501_ KTSQL 2506_ KFTNS 2511_ SNLVA 2516_ FPFPH 2521_ AAILE 2526_ DLDGS 2531_ LSGKN 2536_ RSHIL 2541_ ASMET 2546_ LSASC 2551_ LVNSS 2556_ FGRVV 2561_ HGSAC 2566_ GGGVL 2571_ FHRMS 2576_ IGLAN 2581_ TPEVS 2586_ YDLTM 2591_ TDSRN 2596_ KTTTV 2601_ NYVRD 2606_ TLSNP 2611_ RGWMA 2616_ LLLDQ 2621_ ETYSL 2626_ QSENL 2631_ WINRS 2636_ LQYSA 2641_ TFDNF 2646_ APGNY 2651_ LLLVH 2656_ TDLPP 2661_ YPDIL 2666_ LRCGS 2671_ RVGLS 2676_ FPFLP 2681_ SPGQN 2686_ QGCDW 2691_ FFNSQ 2696_ LRQLT 2701_ YLVSG 2706_ EGQVQ 2711_ VILRV 2716_ KEGMP 2721_ PTISA 2726_ STSAP 2731_ ESALK 2736_ WSLPE 2741_ TWQGV 2746_ EEGWG 2751_ GYNNT 2756_ IPGPG 2761_ DDVLI 2766_ LPNRT 2771_ VLVDT 2776_ DLPFF 2781_ KGLYV 2786_ MGTLD 2791_ FPVDR 2796_ SNVLS 2801_ VACMV 2806_ IAGGE 2811_ LKVGT 2816_ LENPL 2821_ EKEQK 2826_ LLILL 2831_ RASEG 2836_ VFCDR 2841_ MNGIH 2846_ IDPGT 2851_ IGVYG 2856_ KVHLY 2861_ SAYPK 2866_ NSWTH 2871_ LGADI 2876_ ASGNE 2881_ RIIVE 2886_ DAVDW 2891_ RPHDK 2896_ IVLSS 2901_ SSYEP 2906_ HEAEV 2911_ LTVKE 2916_ VKGHH 2921_ VRIYE 2926_ RLKHR 2931_ HIGSV 2936_ HVTED 2941_ GRHIR 2946_ LAAEV 2951_ GLLTR 2956_ NIQIQ 2961_ PDVSC 2966_ RGRLF 2971_ VGSFR 2976_ KSSRE 2981_ EFSGV 2986_ LQLLN 2991_ VEIQN 2996_ FGSPL 3001_ YSSVE 3006_ FSNVS 3011_ AGSWI 3016_ ISSTL 3021_ HQSCG 3026_ GGIHA 3031_ AASHG 3036_ VLLND 3041_ NIVFG 3046_ TAGHG 3051_ IDLEG 3056_ QAYTV 3061_ TNNLV 3066_ VLMTQ 3071_ PAWST 3076_ IWVAG 3081_ IKVNQ 3086_ VKDIN 3091_ LHGNV 3096_ VAGSE 3101_ RLGFH 3106_ IRGHK 3111_ CSSCE 3116_ LLWSD 3121_ NVAHS 3126_ SLHGL 3131_ HLYKE 3136_ SGLDN 3141_ CTRIS 3146_ GFLAF 3151_ KNFDY 3156_ GAMLH 3161_ VENSV 3166_ EIENI 3171_ TLVDN 3176_ TIGLL 3181_ AVVYV 3186_ FSAPQ 3191_ NSVKK 3196_ VQIVL 3201_ RNSVI 3206_ VATSS 3211_ SFDCI 3216_ QDKVK 3221_ PHSAN 3226_ LTSTD 3231_ RAPSN 3236_ PRGGR 3241_ IGILW 3246_ PVFTS 3251_ EPNQW 3256_ PQEPW 3261_ HKVRN 3266_ DHSIS 3271_ GIMKL 3276_ QDVTF 3281_ SSFVK 3286_ SCYSD 3291_ DLDVC 3296_ ILPNA 3301_ ENSGI 3306_ MHPIT 3311_ AERTR 3316_ MLKIK 3321_ DKNKF 3326_ YFPSL 3331_ QPRKD 3336_ LGKVV 3341_ CPELD 3346_ CASPR 3351_ KYLFK 3356_ DLDGR 3361_ ALGLP 3366_ PPVSV 3371_ FPKTE 3376_ AEWTA 3381_ SFFNA 3386_ GTFRE 3391_ EQKCT 3396_ YQFLM 3401_ QGFIC 3406_ KQTDQ 3411_ VVLIL 3416_ DSADA 3421_ IWAIQ 3426_ KLYPV 3431_ VSVTS 3436_ GFVDV 3441_ FSSVN 3446_ ANIPC 3451_ STSGS 3456_ VSTFY 3461_ SILPI 3466_ RQITK 3471_ VCFMD 3476_ QTPQV 3481_ LRFFL 3486_ LGNKS 3491_ TSKLL 3496_ LAVFY 3501_ HELQS 3506_ PHVFL 3511_ GESFI 3516_ PPTLV 3521_ QSASL 3526_ LLNES 3531_ IGANY 3536_ FNIMD 3541_ NLLYV 3546_ VLQGE 3551_ EPIEI 3556_ RSGVS 3561_ IHLAL 3566_ TVMVS 3571_ VLEKG 3576_ WEIVI 3581_ LERLT 3586_ NFLQI 3591_ GQNQI 3596_ RFIHE 3601_ MPGHE 3606_ ETLKA 3611_ IADSR 3616_ AKRKR 3621_ NCPTV 3626_ TCTSH 3631_ YRRVG 3636_ QRRPL 3641_ MMEMN 3646_ SHRAS 3651_ PPMTV 3656_ ETISK 3661_ VIVIE 3666_ IGDSP 3671_ TVRST 3676_ GMISS 3681_ LSSNK 3686_ LQNLA 3691_ HRVIT 3696_ AQQTG 3701_ VLENV 3706_ LNMTI 3711_ GALLV 3716_ TQSKG 3721_ VIGYG 3726_ NTSSF 3731_ KTGNL 3736_ IYIRP 3741_ YALSI 3746_ LVQPS 3751_ DGEVG 3756_ NELPV 3761_ QPQLV 3766_ FLDEQ 3771_ NRRVE 3776_ SLGPP 3781_ SEPWT 3786_ ISASL 3791_ EGASD 3796_ SVLKG 3801_ CTQAE 3806_ TQDGY 3811_ VSFYN 3816_ LAVLI 3821_ SGSNW 3826_ HFIFT 3831_ VTSPP 3836_ GVNFT 3841_ ARSKP 3846_ FAVLP 3851_ VTRKE 3856_ KSTII 3861_ LAASL 3866_ SSVAS 3871_ WLALS 3876_ CLVCC 3881_ WLKRS 3886_ KSRKT 3891_ KPEEI 3896_ PESQT 3901_ NNQNI 3906_ HIHIS 3911_ SKRRE 3916_ SQGPK 3921_ KEDTV 3926_ VGEDM 3931_ RMKVM 3936_ LGKVN 3941_ QCPHQ 3946_ LMNGV 3951_ SRRKV 3956_ SRHIV 3961_ REEEA 3966_ AVPAP 3971_ GTTGI 3976_ TSHGH 3981_ ICAPG 3986_ APAQQ 3991_ VYLQE 3996_ TGNWK 4001_ EGQEQ 4006_ LLRYQ 4011_ LAGQN 4016_ QLLLL 4021_ CPDFR 4026_ QERQQ 4031_ LPGQS 4036_ RLSKQ 4041_ SGSLG 4046_ LSQEK 4051_ KASCG 4056_ ATEAF 4061_ CLHSV 4066_HPETI

Protein annotations

Protein functions:

1: Promotes ciliogenesis in renal epithelial cells and therefore participates in the tubules formation and/ or ensures the maintenance of the architecture of the lumen of the kidney (By similarity). Has an impact on cellular symmetry by ensuring correct bipolar cell division through the regulation of centrosome duplication and mitotic spindle assembly and by maintaining oriented cell division (OCD) during tubular elongation through planar cell polarity (PCP) pathway (PubMed:20554582). During epithelial cell morphogenesis, it also regulates cell-cell and cell-matrix adhesion and participates in cell motility (By similarity). Promotes cell-cell contact through the positive regulation of PTK2 kinase activity leading to either positive regulation of epithelial cell proliferation through the HRAS/RAF1 pathways, or negative regulation of apoptosis through the PDK1/AKT1 pathway (By similarity). May act in collecting-duct and biliary differentiation (PubMed:11919560). May participate in the regulation of the cholangiocytes proliferation and the CCN2 production in an CXCL8-dependent manner (PubMed:30898581)