DYST_MOUSE Q91ZU6
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UniProt:Q91ZU6
Recommended name:Dystonin
EC number:
Alternative names:(Bullous pemphigoid antigen 1) (BPA) (Dystonia musculorum protein) (Hemidesmosomal plaque protein) (Microtubule actin cross-linking factor 2)
Cleaved into:
GeneID:13518
Gene names (primary ):Dst
Gene names (synonym ):Bpag1 Macf2
Gene names (ORF ):
Length:7393
Mass:834218
Sequence:MAGYLSPAAYMYVEEQEYLQAYEDVLERYKDERDKVQKKTFTKWINQHLMKVRKHVNDLYEDLRDGHNLISLLEVLSGDTLPREKGRMRFHRLQNVQIALDYLKRRQVKLVNIRNDDITDGNPKLTLGLIWTIILHFQISDIHVTGESEDMSAKERLLLWTQQATEGYAGVRCENFTTCWRDGKLFNAIIHKYRPDLIDMNTVAVQSNLANLEHAFYVAEKIGVIRLLDPEDVDVSSPDEKSVITYVSSLYDAFPKVPEGGEGIGANDVEVKWIEYQNMVNYLIQWIRHHVVTMSERTFPNNPLELKALYNQYLQFKEKEIPPKEMEKSKIKRLYKLLEIWIEFGRIKLLQGYHPNDIEKEWGKLIIAMLEREKALRPEVERLDMLQQIATRVQRDSVSCEDKLILARNALQSDSKRLESGVQFQNEAEIAGYILECENLLRQHVIDVQILIDGKYYQADQLVQRVAKLRDEIMALRNECSSVYSKGRMLTTEQTKLMISGITQSLNSGFAQTLHPSLNSGLTQSLTPSLTSSSVTSGLSSGMTSRLTPSVTPVYAPGFPSVVAPNFSLGVEPNSLQTLKLMQIRKPLLKSSLLDQNLTEEEVNMKFVQDLLNWVDEMQVQLDRTEWGSDLPSVESHLENHKNVHRAIEEFESSLKEAKISEIQMTAPLKLSYTDKLHRLESQYAKLLNTSRNQERHLDTLHNFVTRATNELIWLNEKEESEVAYDWSERNSSVARKKSYHAELMRELEQKEESIKAVQEIAEQLLLENHPARLTIEAYRAAMQTQWSWILQLCQCVEQHIQENSAYFEFFNDAKEATDYLRNLKDAIQRKYSCDRSSSIHKLEDLVQESMEKEELLQYRSVVAGLMGRAKTVVQLKPRNPDNPLKTSIPIKAICDYRQIEITIYKDDECVLANNSHRAKWKVISPTGNEAMVPSVCFTVPPPNKEAVDFANRIEQQYQSVLTLWHESHINMKSVVSWHYLVNEIDRIRASNVASIKTMLPGEHQQVLSNLQSRLEDFLEDSQESQIFSGSDISQLEKEVSVCRKYYQELLKSAEREEQEESVYNLYISEVRNIRLRLESCEDRLIRQIRTPLERDDLHESMLRITEQEKLKKELDRLKDDLGTITNKCEEFFSQAADSPSVPALRSELSVVIQSLSQIYSMSSTYIEKLKTVNLVLKNTQAAEALVKLYETKLCEEEAVIADKNNIENLMSTLKQWRSEVDEKREVFHALEDELQKAKAISDEMFKTHKERDLDFDWHKEKADQLVERWQSVHVQIDNRLRDLEGIGKSLKHYRDSYHPLDDWIQHIETTQRKIQENQPENSKALALQLNQQKMLVSEIEVKQSKMDECQKYSEQYSAAVKDYELQTMTYRAMVESQQKSPVKRRRIQSSADLVIQEFMDLRTRYTALVTLMTQYIKFAGDSLKRLEEEEKSLDEEKKQHIEKAKELQKWVSNISKTLGDGEKAGKPLFSKQQMSSKEISTKKEQFSEALQTTQIFLAKHGDKLTEEERSDLEKQVKTLQEGYNLLFSESLKQQELQPSGESKVPEKPDKVIAGTINQTTGEVLSVFQAVLRGLIDYETGIRLLEAQLVITGLISPELRKCFDLRDAESHGLIDEQVLRQLKELNRAKQLISTASPTSIPVLDSLAQGMVSESMAIRVLEILLSAGPLLVPATGEHLTLQQAFQQNLISSALFSKVLERQDTCKDLIDPCTSEKVSLTDMVQRSILQENTRMWLLPVRPQEAGRITLKCGRSVSILRAAHEGLIDRETMFRLLGAQLLSGGLIDCNSGQKMTVEEAVAEGVIDRDTASSILTYQVQTGGIVHSNPAKRLTVDEAVQCELITSSSALLVLEAQRGYVGLIWPHSGEIFPTSSSLQQELITNELASKILNGRQKIAALYIPESSQVIGLDAAKQLGIIDNNTASVLKSVTLPDKMPDLGDLEDCKNAKRWLSFCKLQPSTVHDYRQEEGGSDGEEPVTAQSSEQTKKLFLSYLMVNSYMDAHTGQRLLLYDGDLDEAVGMLLESCGTELGADTSTRESLSVLTIPDAFPDCALSEEKHECSAAAAGPDKCHYSHPGHKESLENAKWDMNEAFCKMGNNDSNGELPRPENLADTTVVQKGSESPSRVRVPKPTSSSTQPEGSVLRPESGSILKGCKSQSEPVTKKYPDGANHSHFLTSETSRPCDSNEREDEENIQKGPSVFDYSPRLSALLSHDELRQSQGRFSDTSTPQNTGYLCEASTLSPSDQRVLADQSTREKFQDQFLGIAAISVSLQGAPCGQKPVDTECSSSQVHYHSEESMSDASAESGATRQTDESEKTGSKVEDNSCTMVPGGGSRNDNTSDCGPLSHKGAIDAGDYETSLLAGQQSDTATDSDSDDYFYDTPLFEDEDHDSLILQGDDRDCLQPEDYDTSLQEENDRTPPPDDIFYDVMKEKENPEFPHGGMDESLGVENKVCCPQGFPVGIEKPELYLAGEKEFNSGGSEQLVESVSESENPPGLWDSESDSLTEGEIIGRKERLGASLTPDGHWRGDREECDTSRESQSDTDGVGSIQSSESYRPYMSDGSDLDEEDNGGRSSEDSGDGRGGQGVADEGGEPQYQADPTQLYTAIRKEHGGETQNVSDMIPLDKTHSYSPLETQHGAGVFQPESAGRGGWDTERSSHPELTTEADEEDEASLSTHMATKGVSLSNAEGTASEEIRLVQGPDSTGILKAEDLENVSPEISPSSDNIVRSEAELGGGASEDGHLSFTGSDRDQQGPGRGLVKGRDGQSDKLVDETSIREMGFQKEGVLMSSPEEGGEEERDLEPFPNGSATESLNMGKSQVPPLLTHTEELSHRGAPHTTTMTTTMTLEGEAKNVQTGLTESPVLLETLAEIFDTPASKVTRADLTSAVTASEMKSQVKEDSLTGGPEKETGPCTSLGHCDKCIHVDMLEPNEHTPSCALVAPPTVKDNLCSVNNAGEKSVRPQEDWPPAAEVRLSDACVEESISEGKAGILQFTPENSDSTLSRLPHQSVAGWGKSADSVQARLPVSGVRHTSADTLDVGCPQLESSREKASAEEEPHRERALSLKPQEREHHMLGFVEDGRSILKSSLDKVHMNLQEVGDPSAGTGTKISIQNLIRRAILSELPNEVSNVPSHGISPISNSSEVRAESGGDPFCITSFLHLLKQNQPPQETPGISELAKVLTQMDCDPEQRGLGSELLPPQLKNAFYKLLFDGYATEKDQAEALGQTSCAVPKMAEEKPHVCSDLRNKEGHHCPLNPQAVGEAEVEPFSVHIAALPGGEKLGELCSEPPEHSESTSGSKERSSDSSSKEKCSNGLQQCLQHTEKMHEYLVLLQDMKPPLDNQASVESSLEALKSQLKQLEAFELGLAPIAVFLRKDLKLAEEFLKSFPSDLPRRHHEELSKSHQRLQNAFSSLSSVSSERMKLIKLAINSEMSKLAVRHEDFLHKLTSYSDWVSEKSRSVKAIQTVNVQDTELVKNSVKFLKNVLADLSHTKMQLETTAFDVQSFISDYAQDLSPSQSRQLLRLLNTTQKGFLDLQELVTTEADRLEALLQLEQELGHQKVVAERQQEYREKLQGLCDLLTQTENRLISNQEAFVIGDGTVELQKYQSKQEELQRDMQGSTQAMEEIVRNTELFLKESGDELSQADRALIEQKLNEVKMKCAQLNLKAEQSRKELDKAVTTALKEETEKVAAVRQLEESKTKIENLLNWLSNVEEDSEGVWTKHTQPMEQNGTYLHEGDSKLGAGEEDEVNGNLLETDAEGHSEATKGNLNQQYEKVKAQHGKIMAQHQAVLLATQSAQVLLEKQGHYLSPEEKEKLQKNTQELKVHYEKVLAECEKKVKLTHSLQEELEKFDTDYSEFEHWLQQSEQELANLEAGADDLSGLMDKLTRQKSFSEDVISHKGDLRYITISGNRVIDAAKSCSKRDSDRIGKDSVETSATHREVQTKLDQVTDRFRSLYSKCSVLGNNLKDLVDQYQQYEDASCGLLSGLQACEAKASKHLREPIALDPKNLQRQLEETKALQGQISSQQVAVEKLKKTAEVLLDAKGSLLPAKNDIQKTLDDIVGRYDDLSKCVNERNEKLQITLTRSLSVQDALDEMLDWMGSVESSLVKPGQVPLNSTALQDLISKDTMLEQDITGRQSSINAMNEKVKTFIETTDPSTASSLQAKMKDLSARFSEASQKHKEKLAKMVELKAKVEQFEKLSDKLQTFLETQSQALTEVAMPGKDVPELSQHMQESTAKFLEHRKDLEALHSLLKEISSHGLPGDKALVFEKTNNLSRKFKEMEDTIQEKKDALSSCQEQLSAFQTLAQSLKTWIKETTKQVPVVKPSLGTEDLRKSLEETKKLQEKWNLKAPEIHKANNSGVSLCNLLSALISPAKAIAAAKSGGVILNGEGTDTNTQDFLANKGLTSIKKDMTDISHSYEDLGLLLKDKIVELNTKLSKLQKAQEESSAMMQWLEKMNKTASRWRQTPTPADTESVKLQVEQNKSFEAELKQNVNKVQELKDKLSELLEENPEAPEAQSWKQALAEMDTKWQELNQLTMDRQQKLEESSNNLTQFQTTEAQLKQWLMEKELMVSVLGPLSIDPNMLNTQKQQVQILLQEFDTRKPQYEQLTAAGQGILSRPGEDPSLHGIVNEQLEAVTQKWDNLTGQLRDRCDWIDQAIVKSTQYQSLLRSLSGTLTELDDKLSSGLTSGALPDAVNQQLEAAQRLKQEIEQQAPKIKEAQEVCEDLSALVKEEYLKAELSRQLEGILKSFKDIEQKTENHVQHLQSACASSHQFQQMSKDFQAWLDAKKEEQRDSPPISAKLDVLESLLNSQKDFGKTFTEQSNIYEKTISEGENLLLKTQGAEKAALQLQLNTMKTDWDRFRKQVKEREEKLKDSLEKALKYREQVETLRPWIDRCQHSLDGVTFSLDPTESESSIAELKSLQKEMDHHFGMLELLNNTANSLLSVCEVDKEAVTEENQSLMEKVNRVTEQLQSKTVSLENMAQKFKEFQEVSRDTQRQLQDTKEQLEVHHSLGPQAYSNKHLSVLQAQQKSLQTLKQQVDEAKRLAQDLVVEAADSKGTSDVLLQAETLAEEHSELSQQVDEKCSFLETKLQGLGHFQNTIREMFSQFTECDDELDGMAPVGRDAETLRKQKACMQTFLKKLEALMASNDSANRTCKMMLATEETSPDLIGVKRDLEALSKQCNKLLDRAKTREEQVDGATEKLEEFHRKLEEFSTLLQKAEEHEESQGPVGTETETINQQLDVFKVFQKEEIEPLQVKQQDVNWLGQGLIQSAAANTCTQGLEHDLDSVNSRWKTLNKKVAQRTSQLQEALLHCGRFQDALESLLSWMADTEELVANQKPPSAEFKVVKAQIQEQKLLQRLLEDRKSTVEVIKREGEKIAASAEPADRVKLTRQLSLLDSRWEALLSRAEARNRQLEGISVVAQEFHETLEPLNEWLTAVEKKLANSEPIGTQAPKLEEQISQHKALQEDILLRKQSVDQALLNGLELLKQTTGDEVLIIQDKLEAIKARYKDITKLSADVAKTLEHALQLAGQLQSMHKELCNWLDKVEVELLSYETQGLKGEAASQVQERQKELKNEVRSNKALVDSLNEVSSALLELVPWRAREGLEKTIAEDNERYRLVSDTITQKVEEIDAAILRSQQFEQAADAELSWITETQKKLMSLGDIRLEQDQTSAQLQVQKAFTMDILRHKDIIDELVTSGHKIMTTSSEEEKQSMKKKLDKVLKKYDAVCQINSERHLQLERAQSLVSQFWETYEELWPWLTETQRIISQLPAPALEYETLRRQQEEHRQLRELIAEHKPHIDKMNKTGPQLLELSPKEGIYIQEKYVAADTLYSQIKEDVKKRAVVLDEAISQSTQFHDKIDQILESLERIAERLRQPPSISAEVEKIKEQIGENKSVSVDMEKLQPLYETLRQRGEEMIARSEGTEKDVSARAVQDKLDQMVFIWGSIHTLVEEREAKLLDVMELAEKFWCDHMSLVVTIKDTQDFIRDLEDPGIDPSVVKQQQEAAEAIREEIDGLQEELDMVITLGSELIAACGEPDKPIVKKSIDELNSAWDSLNKAWKDRVDRLEEAMQAAVQYQDGLQGIFDWVDIAGNKLATMSPIGTDLETVKQQIEELKQFKSEAYQQQIEMERLNHQAELLLKKVTEEADKHTVQDPLMELKLIWDSLDERIVSRQHKLEGALLALGQFQHALDELLAWLTHTKGLLSEQKPVGGDPKAIEIELAKHHVLQNDVLAHQSTVEAVNKAGNDLIESSEGEEASNLQYKLRILNQRWQDILEKTDQRKQQLDSALRQAKGFHGEIEDLQQWLTDTERHLLASKPLGGLPETAKEQLNAHMEVCTAFAIKEETYKSLMLRGQQMLARCPRSAETNIDQDITNLKEKWESVKSKLNEKKTKLEEALHLAMNFHNSLQDFINWLTQAEQTLNVASRPSLILDTILFQIDEHKVFANEVNSHREQIIELDKTGTHLKYFSQKQDVVLIKNLLISVQSRWEKVVQRLVERGRSLDEARKRAKQFHEAWSKLMEWLEESEKSLDSELEIANDPDKIKAQLVQHKEFQKSLGGKHSVYDTTNRTGRSLKEKTSLADDNLKLDNMLSELRDKWDTICGKSVERQNKLEEALLFSGQFTDALQALIDWLYRVEPQLAEDQPVHGDIDLVMNLIDNHKVFQKELGKRTSSVQALKRSARELIEGSRDDSSWVRVQMQELSTRWETVCALSISKQTRLESALQQAEEFHSVVHTLLEWLAEAEQTLRFHGALPDDEDALRTLIEQHKEFMKRLEEKRAELSKATGMGDALLAVCHPDSITTIKHWITIIQARFEEVLAWAKQHQQRLAGALAGLIAKQELLETLLAWLQWAETTLTEKDKEVIPQEIEEVKTLIAEHQTFMEEMTRKQPDVDKVTKTYKRRATDPPSLQSHIPVLDKGRAGRKRFPASGFYPSGSQTQIETKNPRVNLLVSKWQQVWLLALERRRKLNDALDRLEELREFANFDFDIWRKKYMRWMNHKKSRVMDFFRRIDKDQDGKITRQEFIDGILSSKFPTSRLEMSAVADIFDRDGDGYIDYYEFVAALHPNKDAYKPITDADKIEDEVTRQVAKCKCAKRFQVEQIGDNKYRFFLGNQFGDSQQLRLVRILRSTVMVRVGGGWMALDEFLVKNDPCRVHHHGSKMLRSESNSSITATQPTLAKGRTNMELREKFILADGASQGMAAFRPRGRRSRPSSRGASPNRSTSASSHACQAASPPVPAAASTPKGTPIQGSKLRLPGYLSGKGFHSGEDSALITTAAARVRTQFAESRKTPSRPGSRAGSKAGSRASSRRGSDASDFDISEIQSVCSDVETVPQTHRPVPRAGSRPSTAKPSKIPTPQRRSPASKLDKSSKR
Tissue specificity:Isoform 1 and 2 are expressed in striated and heart muscle cells. Isoform 5 is expressed in the skin. Isoform 6 is expressed in sensory neural cells of the dorsal root ganglion and with low level in the skin (at protein level). Isoform 1 is expressed predominantly in the brain and spinal cord with low levels in the heart (PubMed:14581450). Isoform 2 is predominantly expressed in muscle and heart and with low levels in the brain. Isoform 5 is expressed in the skin and with low levels in myoblast cells. Isoform 6 is expressed in neurons. Isoform 7 is expressed in lung and with low levels in the brain. {ECO:0000269|PubMed:11514586, ECO:0000269|PubMed:14576348, ECO:0000269|PubMed:14581450, ECO:0000269|PubMed:16797530, ECO:0000269|PubMed:18638474, ECO:0000269|PubMed:19932097, ECO:0000269|PubMed:20209123, ECO:0000269|PubMed:7670468, ECO:0000269|PubMed:7736575, ECO:0000269|PubMed:8752219}.
Induction:
Developmental stage:Isoform 1 is the major form expressed in the dorsal root ganglia at 14.5 dpc. Isoform 2 is predominantly expressed in the myocardium, skeletal muscles, bone cartilage and epithelia of the tongue at 14.5 dpc. Isoform 5 is expressed at high levels in the epidermis and mucosal epithelia of the digestive tracts at 14.5 dpc. {ECO:0000269|PubMed:11514586, ECO:0000269|PubMed:7670468}.
Protein families:Plakin or cytolinker family