Gene name: ATP13A1

Uniprot entry:

Q9HD20

Protein names:

Endoplasmic reticulum transmembrane helix translocase (EC 7.4.2.-) (Endoplasmic reticulum P5A-ATPase)

Protein sequence:

1_MAAAA 6_ AVGNA 11_ VPCGA 16_ RPCGV 21_ RPDGQ 26_ PKPGP 31_ QPRAL 36_ LAAGP 41_ ALIAN 46_ GDELV 51_ AAVWP 56_ YRRLA 61_ LLRRL 66_ TVLPF 71_ AGLLY 76_ PAWLG 81_ AAAAG 86_ CWGWG 91_ SSWVQ 96_ IPEAA 101_ LLVLA 106_ TICLA 111_ HALTV 116_ LSGHW 121_ SVHAH 126_ CALTC 131_ TPEYD 136_ PSKAT 141_ FVKVV 146_ PTPNN 151_ GSTEL 156_ VALHR 161_ NEGED 166_ GLEVL 171_ SFEFQ 176_ KIKYS 181_ YDALE 186_ KKQFL 191_ PVAFP 196_ VGNAF 201_ SYYQS 206_ NRGFQ 211_ EDSEI 216_ RAAEK 221_ KFGSN 226_ KAEMV 231_ VPDFS 236_ ELFKE 241_ RATAP 246_ FFVFQ 251_ VFCVG 256_ LWCLD 261_ EYWYY 266_ SVFTL 271_ SMLVA 276_ FEASL 281_ VQQQM 286_ RNMSE 291_ IRKMG 296_ NKPHM 301_ IQVYR 306_ SRKWR 311_ PIASD 316_ EIVPG 321_ DIVSI 326_ GRSPQ 331_ ENLVP 336_ CDVLL 341_ LRGRC 346_ IVDEA 351_ MLTGE 356_ SVPQM 361_ KEPIE 366_ DLSPD 371_ RVLDL 376_ QADSR 381_ LHVIF 386_ GGTKV 391_ VQHIP 396_ PQKAT 401_ TGLKP 406_ VDSGC 411_ VAYVL 416_ RTGFN 421_ TSQGK 426_ LLRTI 431_ LFGVK 436_ RVTAN 441_ NLETF 446_ IFILF 451_ LLVFA 456_ IAAAA 461_ YVWIE 466_ GTKDP 471_ SRNRY 476_ KLFLE 481_ CTLIL 486_ TSVVP 491_ PELPI 496_ ELSLA 501_ VNTSL 506_ IALAK 511_ LYMYC 516_ TEPFR 521_ IPFAG 526_ KVEVC 531_ CFDKT 536_ GTLTS 541_ DSLVV 546_ RGVAG 551_ LRDGK 556_ EVTPV 561_ SSIPV 566_ ETHRA 571_ LASCH 576_ SLMQL 581_ DDGTL 586_ VGDPL 591_ EKAML 596_ TAVDW 601_ TLTKD 606_ EKVFP 611_ RSIKT 616_ QGLKI 621_ HQRFH 626_ FASAL 631_ KRMSV 636_ LASYE 641_ KLGST 646_ DLCYI 651_ AAVKG 656_ APETL 661_ HSMFS 666_ QCPPD 671_ YHHIH 676_ TEISR 681_ EGARV 686_ LALGY 691_ KELGH 696_ LTHQQ 701_ AREVK 706_ REALE 711_ CSLKF 716_ VGFIV 721_ VSCPL 726_ KADSK 731_ AVIRE 736_ IQNAS 741_ HRVVM 746_ ITGDN 751_ PLTAC 756_ HVAQE 761_ LHFIE 766_ KAHTL 771_ ILQPP 776_ SEKGR 781_ QCEWR 786_ SIDGS 791_ IVLPL 796_ ARGSP 801_ KALAL 806_ EYALC 811_ LTGDG 816_ LAHLQ 821_ ATDPQ 826_ QLLRL 831_ IPHVQ 836_ VFARV 841_ APKQK 846_ EFVIT 851_ SLKEL 856_ GYVTL 861_ MCGDG 866_ TNDVG 871_ ALKHA 876_ DVGVA 881_ LLANA 886_ PERVV 891_ ERRRR 896_ PRDSP 901_ TLSNS 906_ GIRAT 911_ SRTAK 916_ QRSGL 921_ PPSEE 926_ QPTSQ 931_ RDRLS 936_ QVLRD 941_ LEDES 946_ TPIVK 951_ LGDAS 956_ IAAPF 961_ TSKLS 966_ SIQCI 971_ CHVIK 976_ QGRCT 981_ LVTTL 986_ QMFKI 991_ LALNA 996_ LILAY 1001_ SQSVL 1006_ YLEGV 1011_ KFSDF 1016_ QATLQ 1021_ GLLLA 1026_ GCFLF 1031_ ISRSK 1036_ PLKTL 1041_ SRERP 1046_ LPNIF 1051_ NLYTI 1056_ LTVML 1061_ QFFVH 1066_ FLSLV 1071_ YLYRE 1076_ AQARS 1081_ PEKQE 1086_ QFVDL 1091_ YKEFE 1096_ PSLVN 1101_ STVYI 1106_ MAMAM 1111_ QMATF 1116_ AINYK 1121_ GPPFM 1126_ ESLPE 1131_ NKPLV 1136_ WSLAV 1141_ SLLAI 1146_ IGLLL 1151_ GSSPD 1156_ FNSQF 1161_ GLVDI 1166_ PVEFK 1171_ LVIAQ 1176_ VLLLD 1181_ FCLAL 1186_ LADRV 1191_ LQFFL 1196_GTPKL

Protein annotations

Protein functions:

1: Endoplasmic reticulum translocase required to remove mitochondrial transmembrane proteins mistargeted to the endoplasmic reticulum (PubMed:32973005, PubMed:36264797). Acts as a dislocase that mediates the ATP-dependent extraction of mislocalized mitochondrial transmembrane proteins from the endoplasmic reticulum membrane (PubMed:32973005). Specifically binds mitochondrial tail-anchored transmembrane proteins: has an atypically large substrate-binding pocket that recognizes and binds moderately hydrophobic transmembranes with short hydrophilic lumenal domains (PubMed:32973005)