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