DST
Ідентифікатори
Символи
DST , BPA, BPAG1, CATX-15, CATX15, D6S1101, DMH, DT, EBSB2, HSAN6, MACF2, dystonin, BP230, BP240, EBS3
Зовнішні ІД
OMIM : 113810 HomoloGene: 134369 GeneCards: DST
Шаблон експресії
Більше даних
Ортологи
Види
Людина
Миша
Entrez
Ensembl
UniProt
RefSeq (мРНК)
RefSeq (білок)
Локус (UCSC)
Хр. 6: 56.46 – 56.95 Mb
н/д
PubMed search
[1]
[2] Вікідані
DST (англ. Dystonin ) – білок, який кодується однойменним геном, розташованим у людей на короткому плечі 6-ї хромосоми.[3] Довжина поліпептидного ланцюга білка становить 7 570 амінокислот , а молекулярна маса — 860 662[4] .
Послідовність амінокислот
10 20 30 40 50
MAGYLSPAAY LYVEEQEYLQ AYEDVLERYK DERDKVQKKT FTKWINQHLM
KVRKHVNDLY EDLRDGHNLI SLLEVLSGDT LPREKGRMRF HRLQNVQIAL
DYLKRRQVKL VNIRNDDITD GNPKLTLGLI WTIILHFQIS DIHVTGESED
MSAKERLLLW TQQATEGYAG IRCENFTTCW RDGKLFNAII HKYRPDLIDM
NTVAVQSNLA NLEHAFYVAE KIGVIRLLDP EDVDVSSPDE KSVITYVSSL
YDAFPKVPEG GEGIGANDVE VKWIEYQNMV NYLIQWIRHH VTTMSERTFP
NNPVELKALY NQYLQFKETE IPPKETEKSK IKRLYKLLEI WIEFGRIKLL
QGYHPNDIEK EWGKLIIAML EREKALRPEV ERLEMLQQIA NRVQRDSVIC
EDKLILAGNA LQSDSKRLES GVQFQNEAEI AGYILECENL LRQHVIDVQI
LIDGKYYQAD QLVQRVAKLR DEIMALRNEC SSVYSKGRIL TTEQTKLMIS
GITQSLNSGF AQTLHPSLTS GLTQSLTPSL TSSSMTSGLS SGMTSRLTPS
VTPAYTPGFP SGLVPNFSSG VEPNSLQTLK LMQIRKPLLK SSLLDQNLTE
EEINMKFVQD LLNWVDEMQV QLDRTEWGSD LPSVESHLEN HKNVHRAIEE
FESSLKEAKI SEIQMTAPLK LTYAEKLHRL ESQYAKLLNT SRNQERHLDT
LHNFVSRATN ELIWLNEKEE EEVAYDWSER NTNIARKKDY HAELMRELDQ
KEENIKSVQE IAEQLLLENH PARLTIEAYR AAMQTQWSWI LQLCQCVEQH
IKENTAYFEF FNDAKEATDY LRNLKDAIQR KYSCDRSSSI HKLEDLVQES
MEEKEELLQY KSTIANLMGK AKTIIQLKPR NSDCPLKTSI PIKAICDYRQ
IEITIYKDDE CVLANNSHRA KWKVISPTGN EAMVPSVCFT VPPPNKEAVD
LANRIEQQYQ NVLTLWHESH INMKSVVSWH YLINEIDRIR ASNVASIKTM
LPGEHQQVLS NLQSRFEDFL EDSQESQVFS GSDITQLEKE VNVCKQYYQE
LLKSAEREEQ EESVYNLYIS EVRNIRLRLE NCEDRLIRQI RTPLERDDLH
ESVFRITEQE KLKKELERLK DDLGTITNKC EEFFSQAAAS SSVPTLRSEL
NVVLQNMNQV YSMSSTYIDK LKTVNLVLKN TQAAEALVKL YETKLCEEEA
VIADKNNIEN LISTLKQWRS EVDEKRQVFH ALEDELQKAK AISDEMFKTY
KERDLDFDWH KEKADQLVER WQNVHVQIDN RLRDLEGIGK SLKYYRDTYH
PLDDWIQQVE TTQRKIQENQ PENSKTLATQ LNQQKMLVSE IEMKQSKMDE
CQKYAEQYSA TVKDYELQTM TYRAMVDSQQ KSPVKRRRMQ SSADLIIQEF
MDLRTRYTAL VTLMTQYIKF AGDSLKRLEE EEKSLEEEKK EHVEKAKELQ
KWVSNISKTL KDAEKAGKPP FSKQKISSEE ISTKKEQLSE ALQTIQLFLA
KHGDKMTDEE RNELEKQVKT LQESYNLLFS ESLKQLQESQ TSGDVKVEEK
LDKVIAGTID QTTGEVLSVF QAVLRGLIDY DTGIRLLETQ LMISGLISPE
LRKCFDLKDA KSHGLIDEQI LCQLKELSKA KEIISAASPT TIPVLDALAQ
SMITESMAIK VLEILLSTGS LVIPATGEQL TLQKAFQQNL VSSALFSKVL
ERQNMCKDLI DPCTSEKVSL IDMVQRSTLQ ENTGMWLLPV RPQEGGRITL
KCGRNISILR AAHEGLIDRE TMFRLLSAQL LSGGLINSNS GQRMTVEEAV
REGVIDRDTA SSILTYQVQT GGIIQSNPAK RLTVDEAVQC DLITSSSALL
VLEAQRGYVG LIWPHSGEIF PTSSSLQQEL ITNELAYKIL NGRQKIAALY
IPESSQVIGL DAAKQLGIID NNTASILKNI TLPDKMPDLG DLEACKNARR
WLSFCKFQPS TVHDYRQEED VFDGEEPVTT QTSEETKKLF LSYLMINSYM
DANTGQRLLL YDGDLDEAVG MLLEGCHAEF DGNTAIKECL DVLSSSGVFL
NNASGREKDE CTATPSSFNK CHCGEPEHEE TPENRKCAID EEFNEMRNTV
INSEFSQSGK LASTISIDPK VNSSPSVCVP SLISYLTQTE LADISMLRSD
SENILTNYEN QSRVETNERA NECSHSKNIQ NFPSDLIENP IMKSKMSKFC
GVNETENEDN TNRDSPIFDY SPRLSALLSH DKLMHSQGSF NDTHTPESNG
NKCEAPALSF SDKTMLSGQR IGEKFQDQFL GIAAINISLP GEQYGQKSLN
MISSNPQVQY HNDKYISNTS GEDEKTHPGF QQMPEDKEDE SEIEEYSCAV
TPGGDTDNAI VSLTCATPLL DETISASDYE TSLLNDQQNN TGTDTDSDDD
FYDTPLFEDD DHDSLLLDGD DRDCLHPEDY DTLQEENDET ASPADVFYDV
SKENENSMVP QGAPVGSLSV KNKAHCLQDF LMDVEKDELD SGEKIHLNPV
GSDKVNGQSL ETGSERECTN ILEGDESDSL TDYDIVGGKE SFTASLKFDD
SGSWRGRKEE YVTGQEFHSD TDHLDSMQSE ESYGDYIYDS NDQDDDDDDG
IDEEGGGIRD ENGKPRCQNV AEDMDIQLCA SILNENSDEN ENINTMILLD
KMHSCSSLEK QQRVNVVQLA SPSENNLVTE KSNLPEYTTE IAGKSKENLL
NHEMVLKDVL PPIIKDTESE KTFGPASISH DNNNISSTSE LGTDLANTKV
KLIQGSELPE LTDSVKGKDE YFKNMTPKVD SSLDHIICTE PDLIGKPAEE
SHLSLIASVT DKDPQGNGSD LIKGRDGKSD ILIEDETSIQ KMYLGEGEVL
VEGLVEEENR HLKLLPGKNT RDSFKLINSQ FPFPQITNNE ELNQKGSLKK
ATVTLKDEPN NLQIIVSKSP VQFENLEEIF DTSVSKEISD DITSDITSWE
GNTHFEESFT DGPEKELDLF TYLKHCAKNI KAKDVAKPNE DVPSHVLITA
PPMKEHLQLG VNNTKEKSTS TQKDSPLNDM IQSNDLCSKE SISGGGTEIS
QFTPESIEAT LSILSRKHVE DVGKNDFLQS ERCANGLGND NSSNTLNTDY
SFLEINNKKE RIEQQLPKEQ ALSPRSQEKE VQIPELSQVF VEDVKDILKS
RLKEGHMNPQ EVEEPSACAD TKILIQNLIK RITTSQLVNE ASTVPSDSQM
SDSSGVSPMT NSSELKPESR DDPFCIGNLK SELLLNILKQ DQHSQKITGV
FELMRELTHM EYDLEKRGIT SKVLPLQLEN IFYKLLADGY SEKIEHVGDF
NQKACSTSEM MEEKPHILGD IKSKEGNYYS PNLETVKEIG LESSTVWAST
LPRDEKLKDL CNDFPSHLEC TSGSKEMASG DSSTEQFSSE LQQCLQHTEK
MHEYLTLLQD MKPPLDNQES LDNNLEALKN QLRQLETFEL GLAPIAVILR
KDMKLAEEFL KSLPSDFPRG HVEELSISHQ SLKTAFSSLS NVSSERTKQI
MLAIDSEMSK LAVSHEEFLH KLKSFSDWVS EKSKSVKDIE IVNVQDSEYV
KKRLEFLKNV LKDLGHTKMQ LETTAFDVQF FISEYAQDLS PNQSKQLLRL
LNTTQKCFLD VQESVTTQVE RLETQLHLEQ DLDDQKIVAE RQQEYKEKLQ
GICDLLTQTE NRLIGHQEAF MIGDGTVELK KYQSKQEELQ KDMQGSAQAL
AEVVKNTENF LKENGEKLSQ EDKALIEQKL NEAKIKCEQL NLKAEQSKKE
LDKVVTTAIK EETEKVAAVK QLEESKTKIE NLLDWLSNVD KDSERAGTKH
KQVIEQNGTH FQEGDGKSAI GEEDEVNGNL LETDVDGQVG TTQENLNQQY
QKVKAQHEKI ISQHQAVIIA TQSAQVLLEK QGQYLSPEEK EKLQKNMKEL
KVHYETALAE SEKKMKLTHS LQEELEKFDA DYTEFEHWLQ QSEQELENLE
AGADDINGLM TKLKRQKSFS EDVISHKGDL RYITISGNRV LEAAKSCSKR
DGGKVDTSAT HREVQRKLDH ATDRFRSLYS KCNVLGNNLK DLVDKYQHYE
DASCGLLAGL QACEATASKH LSEPIAVDPK NLQRQLEETK ALQGQISSQQ
VAVEKLKKTA EVLLDARGSL LPAKNDIQKT LDDIVGRYED LSKSVNERNE
KLQITLTRSL SVQDGLDEML DWMGNVESSL KEQGQVPLNS TALQDIISKN
IMLEQDIAGR QSSINAMNEK VKKFMETTDP STASSLQAKM KDLSARFSEA
SHKHKETLAK MEELKTKVEL FENLSEKLQT FLETKTQALT EVDVPGKDVT
ELSQYMQEST SEFLEHKKHL EVLHSLLKEI SSHGLPSDKA LVLEKTNNLS
KKFKEMEDTI KEKKEAVTSC QEQLDAFQVL VKSLKSWIKE TTKKVPIVQP
SFGAEDLGKS LEDTKKLQEK WSLKTPEIQK VNNSGISLCN LISAVTTPAK
AIAAVKSGGA VLNGEGTATN TEEFWANKGL TSIKKDMTDI SHGYEDLGLL
LKDKIAELNT KLSKLQKAQE ESSAMMQWLQ KMNKTATKWQ QTPAPTDTEA
VKTQVEQNKS FEAELKQNVN KVQELKDKLT ELLEENPDTP EAPRWKQMLT
EIDSKWQELN QLTIDRQQKL EESSNNLTQF QTVEAQLKQW LVEKELMVSV
LGPLSIDPNM LNTQRQQVQI LLQEFATRKP QYEQLTAAGQ GILSRPGEDP
SLRGIVKEQL AAVTQKWDSL TGQLSDRCDW IDQAIVKSTQ YQSLLRSLSD
KLSDLDNKLS SSLAVSTHPD AMNQQLETAQ KMKQEIQQEK KQIKVAQALC
EDLSALVKEE YLKAELSRQL EGILKSFKDV EQKAENHVQH LQSACASSHQ
FQQMSRDFQA WLDTKKEEQN KSHPISAKLD VLESLIKDHK DFSKTLTAQS
HMYEKTIAEG ENLLLKTQGS EKAALQLQLN TIKTNWDTFN KQVKERENKL
KESLEKALKY KEQVETLWPW IDKCQNNLEE IKFCLDPAEG ENSIAKLKSL
QKEMDQHFGM VELLNNTANS LLSVCEIDKE VVTDENKSLI QKVDMVTEQL
HSKKFCLENM TQKFKEFQEV SKESKRQLQC AKEQLDIHDS LGSQAYSNKY
LTMLQTQQKS LQALKHQVDL AKRLAQDLVV EASDSKGTSD VLLQVETIAQ
EHSTLSQQVD EKCSFLETKL QGIGHFQNTI REMFSQFAEF DDELDSMAPV
GRDAETLQKQ KETIKAFLKK LEALMASNDN ANKTCKMMLA TEETSPDLVG
IKRDLEALSK QCNKLLDRAQ AREEQVEGTI KRLEEFYSKL KEFSILLQKA
EEHEESQGPV GMETETINQQ LNMFKVFQKE EIEPLQGKQQ DVNWLGQGLI
QSAAKSTSTQ GLEHDLDDVN ARWKTLNKKV AQRAAQLQEA LLHCGRFQDA
LESLLSWMVD TEELVANQKP PSAEFKVVKA QIQEQKLLQR LLDDRKSTVE
VIKREGEKIA TTAEPADKVK ILKQLSLLDS RWEALLNKAE TRNRQLEGIS
VVAQQFHETL EPLNEWLTTI EKRLVNCEPI GTQASKLEEQ IAQHKALEDD
IINHNKHLHQ AVSIGQSLKV LSSREDKDMV QSKLDFSQVW YIEIQEKSHS
RSELLQQALC NAKIFGEDEV ELMNWLNEVH DKLSKLSVQD YSTEGLWKQQ
SELRVLQEDI LLRKQNVDQA LLNGLELLKQ TTGDEVLIIQ DKLEAIKARY
KDITKLSTDV AKTLEQALQL ARRLHSTHEE LCTWLDKVEV ELLSYETQVL
KGEEASQAQM RPKELKKEAK NNKALLDSLN EVSSALLELV PWRAREGLEK
MVAEDNERYR LVSDTITQKV EEIDAAILRS QQFDQAADAE LSWITETEKK
LMSLGDIRLE QDQTSAQLQV QKTFTMEILR HKDIIDDLVK SGHKIMTACS
EEEKQSMKKK LDKVLKNYDT ICQINSERYL QLERAQSLVN QFWETYEELW
PWLTETQSII SQLPAPALEY ETLRQQQEEH RQLRELIAEH KPHIDKMNKT
GPQLLELSPG EGFSIQEKYV AADTLYSQIK EDVKKRAVAL DEAISQSTQF
HDKIDQILES LERIVERLRQ PPSISAEVEK IKEQISENKN VSVDMEKLQP
LYETLKQRGE EMIARSGGTD KDISAKAVQD KLDQMVFIWE NIHTLVEERE
AKLLDVMELA EKFWCDHMSL IVTIKDTQDF IRDLEDPGID PSVVKQQQEA
AETIREEIDG LQEELDIVIN LGSELIAACG EPDKPIVKKS IDELNSAWDS
LNKAWKDRID KLEEAMQAAV QYQDGLQAVF DWVDIAGGKL ASMSPIGTDL
ETVKQQIEEL KQFKSEAYQQ QIEMERLNHQ AELLLKKVTE ESDKHTVQDP
LMELKLIWDS LEERIINRQH KLEGALLALG QFQHALDELL AWLTHTEGLL
SEQKPVGGDP KAIEIELAKH HVLQNDVLAH QSTVEAVNKA GNDLIESSAG
EEASNLQNKL EVLNQRWQNV LEKTEQRKQQ LDGALRQAKG FHGEIEDLQQ
WLTDTERHLL ASKPLGGLPE TAKEQLNVHM EVCAAFEAKE ETYKSLMQKG
QQMLARCPKS AETNIDQDIN NLKEKWESVE TKLNERKTKL EEALNLAMEF
HNSLQDFINW LTQAEQTLNV ASRPSLILDT VLFQIDEHKV FANEVNSHRE
QIIELDKTGT HLKYFSQKQD VVLIKNLLIS VQSRWEKVVQ RLVERGRSLD
DARKRAKQFH EAWSKLMEWL EESEKSLDSE LEIANDPDKI KTQLAQHKEF
QKSLGAKHSV YDTTNRTGRS LKEKTSLADD NLKLDDMLSE LRDKWDTICG
KSVERQNKLE EALLFSGQFT DALQALIDWL YRVEPQLAED QPVHGDIDLV
MNLIDNHKAF QKELGKRTSS VQALKRSARE LIEGSRDDSS WVKVQMQELS
TRWETVCALS ISKQTRLEAA LRQAEEFHSV VHALLEWLAE AEQTLRFHGV
LPDDEDALRT LIDQHKEFMK KLEEKRAELN KATTMGDTVL AICHPDSITT
IKHWITIIRA RFEEVLAWAK QHQQRLASAL AGLIAKQELL EALLAWLQWA
ETTLTDKDKE VIPQEIEEVK ALIAEHQTFM EEMTRKQPDV DKVTKTYKRR
AADPSSLQSH IPVLDKGRAG RKRFPASSLY PSGSQTQIET KNPRVNLLVS
KWQQVWLLAL ERRRKLNDAL DRLEELREFA NFDFDIWRKK YMRWMNHKKS
RVMDFFRRID KDQDGKITRQ EFIDGILSSK FPTSRLEMSA VADIFDRDGD
GYIDYYEFVA ALHPNKDAYK PITDADKIED EVTRQVAKCK CAKRFQVEQI
GDNKYRFFLG NQFGDSQQLR LVRILRSTVM VRVGGGWMAL DEFLVKNDPC
RVHHHGSKML RSESNSSITT TQPTIAKGRT NMELREKFIL ADGASQGMAA
FRPRGRRSRP SSRGASPNRS TSVSSQAAQA ASPQVPATTT PKGTPIQGSK
LRLPGYLSGK GFHSGEDSGL ITTAAARVRT QFADSKKTPS RPGSRAGSKA
GSRASSRRGS DASDFDISEI QSVCSDVETV PQTHRPTPRA GSRPSTAKPS
KIPTPQRKSP ASKLDKSSKR
Кодований геном білок за функціями належить до м'язових білків , фосфопротеїнів .
Задіяний у таких біологічних процесах, як клітинна адгезія, альтернативний сплайсинг .
Білок має сайт для зв'язування з молекулою актину , іонами металів, іоном кальцію .
Локалізований у клітинній мембрані , цитоплазмі , цитоскелеті , ядрі , мембрані, клітинних контактах, ендоплазматичному ретикулумі , клітинних відростках.
Література
Elgart G.W., Stanley J.R. (1993). Cloning of the 5' mRNA for the 230-kD bullous pemphigoid antigen by rapid amplification of cDNA ends . J. Invest. Dermatol . 101 : 244—246. PMID 8345227 DOI :10.1111/1523-1747.ep12365083
Okumura M., Yamakawa H., Ohara O., Owaribe K. (2002). Novel alternative splicings of BPAG1 (bullous pemphigoid antigen 1) including the domain structure closely related to MACF (microtubule actin cross-linking factor). J. Biol. Chem . 277 : 6682—6687. PMID 11751855 DOI :10.1074/jbc.M109209200
Brown A., Dalpe G., Mathieu M., Kothary R. (1995). Cloning and characterization of the neural isoforms of human dystonin. Genomics . 29 : 777—780. PMID 8575775 DOI :10.1006/geno.1995.9936
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res . 14 : 2121—2127. 2004. PMID 15489334 DOI :10.1101/gr.2596504
Stanley J.R., Tanaka T., Mueller S., Klaus-Kovtun V., Roop D. (1988). Isolation of complementary DNA for bullous pemphigoid antigen by use of patients' autoantibodies . J. Clin. Invest . 82 : 1864—1870. PMID 2461961 DOI :10.1172/JCI113803
Hopkinson S.B., Jones J.C. (1994). Identification of a second protein product of the gene encoding a human epidermal autoantigen. Biochem. J . 300 : 851—857. PMID 8010969 DOI :10.1042/bj3000851
Примітки
Див. також