Рис. MPS III A int
Нуклеотидная и аминокислотная последовательности
гена гепарин-N-сульфатазы
(N-sulfoglucosamine sulfohydrolase (sulfamidase)
gaattccgggcc
atg agc tgc ccc gtg ccc gcc tgc tgc gcg ctg ctg cta gtc ctg
met ser cys pro val pro ala cys cys ala ;eu leu leu val leu 15
ggg ctc tgc cgg gcg cgt ccc cgg aac gca ctg ctg ctc ctc gcg
gly leu arg ala arg pro arg asn ala leu leu leu leu leu ala 30
gat gac gga ggc ttt gag agt ggc gcg tac aac aac agc gcc atc
asp asp gly gly phe glu ser gly ala tyr asn asn ser ala ile 45
gcc acc ccg cac ctg gac gcc ttg gcc cgc cgc agc ctc ctc ttt
ala thr pro his leu asp ala leu ala arg arg ser leu leu phe 60
cgc aat gcc ttc acc tcg gtc agc agc tgc tct ccc agc cgc gcc
arg asn ala phe thr ser val ser ser cys ser pro ser arg ala 75
agc ctc ctc act ggc ctg ccc cag cat cag aat ggg atg tac ggg
ser leu leu thr gly leu pro gln his gln asn gly met tyr gly 90
ctg cac cag gac gtg cac cac ttc aac tcc ttc gac aag gtg cgg
leu his gln asp val his his phe asn ser phe asp lys val arg 105
agc ctg ccg ctg ctg ctc agc caa gct ggt gtg cgc aca ggc atc
ser leu pro leu leu leu ser gln ala gly val arg thr gly ile 120
atc ggg aag aag cac gtg ggg ccg gag acc gtg tac ccg ttt gac
ile gly lys lys his val gly pro glu the val tyr pro phe asp 135
ttt gcg tac acg gag gag aat ggc tcc gtc ctc cag gtg ggg cgg
phe ala tyr thr glu glu asn gly ser val leu gln val gly arg 150
aac atc act aga att aag ctg ctc gtc cgg aaa ttc ctg cag act
asn ile thr arg ile lys leu leu val arg lys phe leu gln thr 165
cag gat gac cgg cct ttc ttc ctc tac gtc gcc ttc cac gac ccc
gln asp asp arg pro phe phe leu tyr val ala phe his asp pro 180
cac cgc tgt ggg cac tcc cag ccc cag tac gga acc ttc tgt gag
his arg cys gly his ser gln pro gln tyr gly thr phe cys glu 195
aag ttt ggc aac gga gag agc ggc atg ggt cgt atc cca gac tgg
lys phe gly asn gly glu ser gly met gly arg ile pro asp trp 205
acc ccc cag gcc tac gac cca ctg gac gtg ctg gtg cct tac ttc
thr pro gln ala tyr asp pro leu asp val leu val pro tyr phe 220
gtc ccc aac acc ccg gca gcc cga gcc gac ctg gcc gct cag tac
val pro asn thr pro ala ala arg ala asp leu ala ala gln tyr 235
acc acc gtc ggc cgc atg gac caa gga gtt gga ctg gtg ctc cag
thr thr val gly arg met asp gln gly val gly leu val leu gln 250
gag ctg cgt gac gcc ggt gtc ctg aac gac aca ctg gtg atc ttc
glu leu arg asp ala gly val leu asn asp thr leu val ile phe 265
acg tcc gac aac ggg atc ccc ttc ccc agc ggc agg acc aac ctg
thr ser asp asn gly ile pro phe pro ser gly arg thr asn leu 280
tac tgg ccg ggc act gct gaa ccc tta ctg gtg tca tcc ccg gag
tyr trp pro gly thr ala glu pro leu leu val ser ser pro glu 295
cac cca aaa cgc tgg ggc caa gtc agc gag gcc tac gtg agc ctc
his pro lys arg trp gly gln val ser glu ala tyr val ser leu 310
cta gac ctc acg ccc acc atc ttg gat tgg ttc tcg atc ccg tac
leu asp leu thr pro thr ile leu asp trp phe ser ile pro tyr 325
ccc agc tac gcc atc ttt ggc tcg aag acc atc cac ctc act ggc
pro ser tyr ala ile phe gly ser lys thr ile his leu thr gly 340
cgg tcc ctc ctg ccg gcg ctg gag gcc gag ccc ctc tgg gcc acc
arg ser leu leu pro ala leu glu ala glu pro leu trp ala thr 355
gtc ttt ggc agc cag agc cac cac gag gtc acc atg tcc tac ccc
val phe gly ser gln ser his his glu val thr met ser tyr pro 370
atg cgc tcc gtg cag cac cgg cac ttc cgc ctc gtg cac aac ctc
met arg set val gln his arg his phe arg leu val his asn leu 385
aac ttc aag atg ccc ttt ccc atc gac cag gac ttc tac gtc tca
asn phe lys met pro phe pro ile asp gln asp phe tyr val ser 400
ccc acc ttc cag gac ctc ctg aac cgc acc aca gct ggt cag ccc
pro thr phe gln asp leu leu asn arg thr thr ala gly gln pro 415
acg ggc tgg tac aag gac ctc cgt cat tac tac tac cgg gcg cgc
thr gly trp tyr lys asp leu arg his tyr tyr tyr arg ala arg 430
tgg gag ctc tac gac cgg agc cgg gac ccc cac gag acc cag aac
trp glu leu tyr asp arg ser arg asp phe his glu thr gln asn 445
ctg gcc acc gac ccg cgc ttt gct cag ctt ctg gag atg ctt cgg
leu ala thr asp pro arg phe ala gln leu leu glu met leu arg 460
gac cag ctg gcc aag tgg cag tgg gag acc cac gac ccc tgg gtg
asp gln leu ala lys trp gln trp glu trp his asp pro trp val 475
tgc gcc ccc gac ggc gtc ctg gag gag aag ctc tct ccc cag tgc
cys ala pro asp gly val leu glu glu lys leu ser pro gln cys 490
cag ccc ctc cac aat gag ctg tga ccatcccaggaggcctgtgcacacatc
gln pro leu his asn glu leu ter
ccaggcatgtcccagacacatcccacacgtgtccgtgtggccggccagcctggggagta
gtggcaacagcccttccgtccacactcccatccaaggagggttcttccttcctgtgggg
tcactcttgccattgcctggagggggaccagagcatgtgaccagagcatgtgcccagcc
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