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
- ↑ Human PubMed Reference:.
- ↑ Mouse PubMed Reference:.
- ↑ HUGO Gene Nomenclature Commitee, HGNC:1090 (англ.) . Архів оригіналу за 10 травня 2017. Процитовано 6 вересня 2017. [Архівовано 2017-05-10 у Wayback Machine.]
- ↑ UniProt, Q03001 (англ.) . Архів оригіналу за 2 вересня 2017. Процитовано 6 вересня 2017.
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