Рис. FBN seq
Нуклеотидная и аминокислотная последовательности гена фибриллина (FBN1) человека ATG CGT CGA GGG CGT CTG CTG GAG ATC GCC CTG GGA TTT ACC GTG met arg arg gly arg leu leu glu ile ala leu gly phe thr val 15 CTT TTA GCG TCC TAC ACG AGC CAT GGG GCG GAC GCC AAT TTG GAG leu leu ala ser tyr thr ser his gly ala asp ala asn leu glu 30 GCT GGG AAC GTG AAG GAA ACC AGA GCC AGT CGG GCC AAG AGA AGA ala gly asn val lys glu thr arg ala ser arg ala lys arg arg 45 GGC GGT GGA GGA CAC GAC GCG CTT AAA GGA CCC AAT GTC TGT GGA gly gly gly gly his asp ala leu lys gly pro asn val cys gly 60 TCA CGT TAT AAT GCT TAC TGT TGC CCT GGA TGG AAA ACC TTA CCT ser arg tyr asn ala tyr cys cys pro gly trp lys thr leu pro 75 GGC GGA AAT CAG TGT ATT GTC CCC ATT TGC CGG CAT TCC TGT GGG gly gly asn gln cys ile val pro ile cys arg his ser cys gly 90 GAT GGA TTT TGT TCG AGG CCA AAT ATG TGC ACT TGC CCA TCT GGT asp gly phe cys ser arg pro asn met cys thr cys pro ser gly 105 CAG ATA GCT CCT TCC TGT GGC TCC AGA TCC ATA CAA CAC TGC AAT gln ile ala pro ser cys gly ser arg ser ile gln his cys asn 120 ATT CGC TGT ATG AAT GGA GGT AGC TGC AGT GAC GAT CAC TGT CTA ile arg cys met asn gly gly ser cys ser asp asp his cys leu 135 TGC CAG AAA GGA TAC ATA GGG ACT CAC TGT GGA CAA CCT GTT TGT cys gln lys gly tyr ile gly thr his cys gly gln pro val cys 150 GAA AGT GGC TGT CTC AAT GGA GGA AGG TGT GTG GCC CCA AAT CGA glu ser gly cys leu asn gly gly arg cys val ala pro asn arg 165 TGT GCA TGC ACT TAC GGA TTT ACT GGA CCC CAG TGT GAA AGA GAT cys ala cys thr tyr gly phe thr gly pro gln cys glu arg asp 180 TAC AGG ACA GGC CCA TGT TTT ACT GTG ATC AGC AAC CAG ATG TGC tyr arg thr gly pro cys phe thr val ile ser asn gln met cys 195 CAG GGA CAA CTC AGC GGG ATT GTC TGC ACA AAA CAG CTC TGC TGT gln gly gln leu ser gly ile val cys thr lys gln leu cys cys 210 GCC ACA GTC GGC CGA GCC TGG GGC CAC CCC TGT GAG ATG TGT CCT ala thr val gly arg ala trp gly his pro cys glu met cys pro 225 GCC CAG CCT CAC CCC TGC CGC CGT GGC TTC ATT CCA AAT ATC CGC ala gln pro his pro cys arg arg gly phe ile pro asn ile arg 240 ACG GGA GCT TGT CAA GAT GTG GAT GAA TGC CAG GCC ATC CCC GGG thr gly ala cys gln asp val asp glu cys gln ala ile pro gly 255 CTC TGT CAG GGA GGA AAT TGC ATT AAT ACT GTT GGG TCT TTT GAG leu cys gln gly gly asn cys ile asn thr val gly ser phe glu 270 TGC AAA TGC CCT GCT GGA CAC AAA CTT AAT GAA GTG TCA CAA AAA cys lys cys pro ala gly his lys leu asn glu val ser gln lys 285 TGT GAA GAT ATT GAT GAA TGC AGC ACC ATT CCT GGA ATC TGT GAA cys glu asp ile asp glu cys ser thr ile pro gly ile cys glu 300 GGG GGT GAA TGT ACA AAC ACA GTC AGC AGT TAC TTT TGC AAA TGT gly gly glu cys thr asn thr val ser ser tyr phe cys lys cys 315 CCC CCT GGT TTT TAC ACC TCT CCA GAT GGT ACC AGA TGC ATA GAT pro pro gly phe tyr thr ser pro asp gly thr arg cys ile asp 330 GTT CGC CCA GGA TAC TGT TAC ACA GCT CTG ACA AAC GGG CGC TGC val arg pro gly tyr cys tyr thr ala leu thr asn gly arg cys 345 TCT AAC CAG CTG CCA CAG TCC ATA ACC AAA ATG CAG TGC TGC TGT ser asn gln leu pro gln ser ile thr lys met gln cys cys cys 360 GAT GCC GGC CGA TGC TGG TCT CCA GGG GTC ACT GTC GCC CCT GAG asp ala gly arg cys trp ser pro gly val thr val ala pro glu 375 ATG TGT CCC ATC AGA GCA ACC GAG GAT TTC AAC AAG CTG TGC TCT met cys pro ile arg ala thr glu asp phe asn lys leu cys ser 390 GTT CCT ATG GTA ATT CCT GGG AGA CCA GAA TAT CCT CCC CCA CCC val pro met val ile pro gly arg pro glu tyr pro pro pro pro 405 CTT GGC CCC ATT CCT CCA GTT CTC CCT GTT CCT CCT GGC TTT CCT leu gly pro ile pro pro val leu pro val pro pro gly phe pro 420 CCT GGA CCT CAA ATT CCG GTC CCT CGA CCA CCA GTG GAA TAT CTG pro gly pro gln ile pro val pro arg pro pro val glu tyr leu 435 TAT CCA TCT CGG GAG CCA CCA AGG GTG CTG CCA GTA AAC GTT ACT tyr pro ser arg glu pro pro arg val leu pro val asn val thr 450 GAT TAC TGC CAG TTG GTC CGC TAT CTC TGT CAA AAT GGA CGC TGC asp tyr cys gln leu val arg tyr leu cys gln asn gly arg cys 465 ATT CCA ACT CCT GGG AGT TAC CGG TGT GAG TGC AAC AAA GGG TTC ile pro thr pro gly ser tyr arg cys glu cys asn lys gly phe 480 CAG CTG GAC CTC CGT GGG GAG TGT ATT GAT GTT GAT GAA TGT GAG gln leu asp leu arg gly glu cys ile asp val asp glu cys glu 495 AAA AAC CCC TGT GCT GGT GGT GAG TGT ATT AAC AAC CAG GGT TCG lys asn pro cys ala gly gly glu cys ile asn asn gln gly ser 510 TAC ACC TGT CAG TGC CGA GCT GGA TAT CAG AGC ACA CTC ACG CGG tyr thr cys gln cys arg ala gly tyr gln ser thr leu thr arg 525 ACA GAA TGC CGA GAC ATT GAT GAG TGT TTA CAG AAT GGC CGG ATC thr glu cys arg asp ile asp glu cys leu gln asn gly arg ile 540 TGC AAT AAT GGA CGC TGC ATC AAC ACA GAT GGC AGT TTT CAT TGC cys asn asn gly arg cys ile asn thr asp gly ser phe his cys 555 GTG TGT AAT GCG GGC TTT CAT GTT ACA CGA GAT GGG AAG AAC TGT val cys asn ala gly phe his val thr arg asp gly lys asn cys 570 GAA GAT ATG GAT GAA TGC AGC ATA AGG AAC ATG TGC CTT AAT GGA glu asp met asp glu cys ser ile arg asn met cys leu asn gly 585 ATG TGT ATC AAT GAA GAT GGC AGT TTT AAA TGT ATT TGC AAA CCT met cys ile asn glu asp gly ser phe lys cys ile cys lys pro 600 GGA TTC CAG CTG GCA TCA GAT GGA CGT TAT TGC AAA GAC ATT AAC gly phe gln leu ala ser asp gly arg tyr cys lys asp ile asn 615 GAG TGT GAA ACC CCT GGG ATC TGC ATG AAT GGG CGT TGC GTC AAC glu cys glu thr pro gly ile cys met asn gly arg cys val asn 630 ACT GAT GGC TCC TAC AGA TGT GAA TGC TTC CCT GGA CTG GCT GTG thr asp gly ser tyr arg cys glu cys phe pro gly leu ala val 645 GGT CTG GAT GGC CGT GTG TGT GTT GAC ACA CAC ATG CGG AGC ACA gly leu asp gly arg val cys val asp thr his met arg ser thr 660 TGC TAT GGT GGA TAC AAG AGA GGC CAG TGT ATC AAA CCT TTG TTT cys tyr gly gly tyr lys arg gly gln cys ile lys pro leu phe 675 GGT GCT GTC ACT AAA TCT GAA TGC TGT TGC GCC AGC ACT GAG TAT gly ala val thr lys ser glu cys cys cys ala ser thr glu tyr 690 GCA TTT GGG GAA CCT TGC CAG CCG TGT CCT GCA CAG AAT TCA GCG ala phe gly glu pro cys gln pro cys pro ala gln asn ser ala 705 GAA TAT CAG GCA CTC TGC AGC AGT GGG CCA GGA ATG ACG TCA GCA glu tyr gln ala leu cys ser ser gly pro gly met thr ser ala 720 GGC AGT GAT ATA AAT GAA TGT GCA CTA GAT CCT GAT ATT TGC CCA gly ser asp ile asn glu cys ala leu asp pro asp ile cys pro 735 AAT GGA ATC TGT GAA AAC CTT CGT GGG ACC TAT AAA TGT ATA TGC asn gly ile cys glu asn leu arg gly thr tyr lys cys ile cys 750 AAT TCA GGA TAT GAA GTG GAT TCA ACT GGG AAA AAC TGC GTT GAT asn ser gly tyr glu val asp ser thr gly lys asn cys val asp 765 ATT AAT GAA TGT GTA CTG AAC AGT CTC CTT TGT GAC AAT GGA CAA ile asn glu cys val leu asn ser leu leu cys asp asn gly gln 780 TGT AGA AAT ACT CCT GGA AGT TTT GTC TGT ACC TGC CCC AAG GGA cys arg asn thr pro gly ser phe val cys thr cys pro lys gly 795 TTT ATC TAC AAA CCT GAT CTA AAA ACA TGT GAA GAC ATT GAT GAA phe ile tyr lys pro asp leu lys thr cys glu asp ile asp glu 810 TGC GAA TCA AGT CCT TGC ATT AAT GGA GTC TGC AAG AAC AGC CCA cys glu ser ser pro cys ile asn gly val cys lys asn ser pro 825 GGC TCT TTT ATT TGT GAA TGT TCT TCT GAA AGT ACT TTG GAT CCA gly ser phe ile cys glu cys ser ser glu ser thr leu asp pro 840 ACA AAA ACC ATC TGC ATA GAA ACC ATC AAG GGC ACT TGC TGG CAG thr lys thr ile cys ile glu thr ile lys gly thr cys trp gln 855 ACT GTC ATT GAT GGG CGA TGT GAG ATC AAC ATC AAT GGA GCC ACC thr val ile asp gly arg cys glu ile asn ile asn gly ala thr 870 TTA AAG TCC CAG TGC TGC TCC TCC CTC GGT GCT GCG TGG GGA AGC leu lys ser gln cys cys ser ser leu gly ala ala trp gly ser 885 CCG TGC ACC CTA TGC CAA GTT GAT CCC ATA TGT GGT AAA GGG TAC pro cys thr leu cys gln val asp pro ile cys gly lys gly tyr 900 TCA AGA ATT AAA GGA ACA CAA TGT GAA GAT ATA GAT GAA TGT GAA ser arg ile lys gly thr gln cys glu asp ile asp glu cys glu 915 GTG TTC CCA GGA GTG TGT AAA AAT GGC CTG TGT GTT AAC ACT AGG val phe pro gly val cys lys asn gly leu cys val asn thr arg 930 GGG TCA TTC AAG TGT CAG TGT CCC AGT GGA ATG ACT TTG GAT GCC gly ser phe lys cys gln cys pro ser gly met thr leu asp ala 945 ACA GGA AGG ATC TGT CTT GAT ATC CGC CTG GAA ACC TGC TTC CTG thr gly arg ile cys leu asp ile arg leu glu thr cys phe leu 960 AGG TAC GAG GAC GAG GAG TGC ACC CTG CCT ATT GCT GGC CGC CAC arg tyr glu asp glu glu cys thr leu pro ile ala gly arg his 975 CGC ATG GAC GCC TGC TGC TGC TCC GTC GGG GCA GCC TGG GGT ACT arg met asp ala cys cys cys ser val gly ala ala trp gly thr 990 GAG GAA TGC GAG GAG TGT CCC ATG AGA AAT ACT CCT GAG TAC GAG glu glu cys glu glu cys pro met arg asn thr pro glu tyr glu 1005 GAG CTG TGT CCG AGA GGA CCC GGA TTT GCC ACA AAA GAA ATT ACA glu leu cys pro arg gly pro gly phe ala thr lys glu ile thr 1020 AAT GGA AAG CCT TTC TTC AAA GAT ATC AAT GAG TGC AAG ATG ATA asn gly lys pro phe phe lys asp ile asn glu cys lys met ile 1035 CCC AGC CTC TGC ACC CAC GGC AAG TGC AGA AAC ACC ATT GGC AGC pro ser leu cys thr his gly lys cys arg asn thr ile gly ser 1050 TTT AAG TGC AGG TGT GAC AGC GGC TTT GCT CTT GAT TCT GAA GAA phe lys cys arg cys asp ser gly phe ala leu asp ser glu glu 1065 AGG AAC TGC ACA GAC ATT GAC GAA TGC CGC ATA TCT CCT GAC CTC arg asn cys thr asp ile asp glu cys arg ile ser pro asp leu 1080 TGT GGC AGA GGC CAG TGT GTG AAC ACC CCT GGG GAC TTT GAA TGC cys gly arg gly gln cys val asn thr pro gly asp phe glu cys 1095 AAG TGT GAC GAA GGC TAT GAA AGT GGA TTC ATG ATG ATG AAG AAC lys cys asp glu gly tyr glu ser gly phe met met met lys asn 1110 TGC ATG GAT ATT GAT GAG TGT CAG AGA GAT CCT CTC CTA TGC CGA cys met asp ile asp glu cys gln arg asp pro leu leu cys arg 1125 GGT GGT GTT TGC CAT AAC ACA GAG GGA AGT TAC CGC TGT GAA TGC gly gly val cys his asn thr glu gly ser tyr arg cys glu cys 1140 CCG CCT GGC CAT CAG CTG TCC CCC AAC ATC TCC GCG TGT ATC GAC pro pro gly his gln leu ser pro asn ile ser ala cys ile asp 1155 ATC AAT GAA TGT GAG CTG AGT GCA CAC CTG TGC CCC AAT GGC CGT ile asn glu cys glu leu ser ala his leu cys pro asn gly arg 1170 TGC GTG AAC CTC ATA GGG AAG TAT CAG TGT GCC TGC AAC CCT GGC cys val asn leu ile gly lys tyr gln cys ala cys asn pro gly 1185 TAC CAT TCA ACT CCC GAT AGG CTA TTT TGT GTT GAC ATT GAT GAA tyr his ser thr pro asp arg leu phe cys val asp ile asp glu 1200 TGC AGC ATA ATG AAT GGT GGT TGT GAA ACC TTC TGC ACA AAC TCT cys ser ile met asn gly gly cys glu thr phe cys thr asn ser 1215 GAA GGC AGC TAT GAA TGT AGC TGT CAG CCG GGA TTT GCA CTA ATG glu gly ser tyr glu cys ser cys gln pro gly phe ala leu met 1230 CCT GAC CAG AGA TCA TGC ACC GAC ATC GAT GAG TGT GAA GAT AAT pro asp gln arg ser cys thr asp ile asp glu cys glu asp asn 1245 CCC AAT ATC TGT GAT GGT GGT CAG TGC ACA AAT ATC CCT GGA GAG pro asn ile cys asp gly gly gln cys thr asn ile pro gly glu 1260
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