                         SEQUENCE LISTING

<110>  Academia Sinica
 
<120>  ENGINEERED ENZYME FOR ENZYME REPLACEMENT THERAPY

<130>  218048-0272PCT

<150>  US 62/201,889
<151>  2015-08-06

<160>  4     

<170>  PatentIn version 3.5

<210>  1
<211>  1956
<212>  DNA
<213>  Homo sapiens beta-glucuronidase


<220>
<221>  CDS
<222>  (1)..(1956)

<400>  1
atg gcc cgg ggg tcg gcg gtt gcc tgg gcg gcg ctc ggg ccg ttg ttg         48
Met Ala Arg Gly Ser Ala Val Ala Trp Ala Ala Leu Gly Pro Leu Leu           
1               5                   10                  15                

tgg ggc tgc gcg ctg ggg ctg cag ggc ggg atg ctg tac ccc cag gag         96
Trp Gly Cys Ala Leu Gly Leu Gln Gly Gly Met Leu Tyr Pro Gln Glu           
            20                  25                  30                    

agc ccg tcg cgg gag tgc aag gag ctg gac ggc ctc tgg agc ttc cgc        144
Ser Pro Ser Arg Glu Cys Lys Glu Leu Asp Gly Leu Trp Ser Phe Arg           
        35                  40                  45                        

gcc gac ttc tct gac aac cga cgc cgg ggc ttc gag gag cag tgg tac        192
Ala Asp Phe Ser Asp Asn Arg Arg Arg Gly Phe Glu Glu Gln Trp Tyr           
    50                  55                  60                            

cgg cgg ccg ctg tgg gag tca ggc ccc acc gtg gac atg cca gtt ccc        240
Arg Arg Pro Leu Trp Glu Ser Gly Pro Thr Val Asp Met Pro Val Pro           
65                  70                  75                  80            

tcc agc ttc aat gac atc agc cag gac tgg cgt ctg cgg cat ttt gtc        288
Ser Ser Phe Asn Asp Ile Ser Gln Asp Trp Arg Leu Arg His Phe Val           
                85                  90                  95                

ggc tgg gtg tgg tac gaa cgg gag gtg atc ctg ccg gag cga tgg acc        336
Gly Trp Val Trp Tyr Glu Arg Glu Val Ile Leu Pro Glu Arg Trp Thr           
            100                 105                 110                   

cag gac ctg cgc aca aga gtg gtg ctg agg att ggc agt gcc cat tcc        384
Gln Asp Leu Arg Thr Arg Val Val Leu Arg Ile Gly Ser Ala His Ser           
        115                 120                 125                       

tat gcc atc gtg tgg gtg aat ggg gtc gac acg cta gag cat gag ggg        432
Tyr Ala Ile Val Trp Val Asn Gly Val Asp Thr Leu Glu His Glu Gly           
    130                 135                 140                           

ggc tac ctc ccc ttc gag gcc gac atc agc aac ctg gtc cag gtg ggg        480
Gly Tyr Leu Pro Phe Glu Ala Asp Ile Ser Asn Leu Val Gln Val Gly           
145                 150                 155                 160           

ccc ctg ccc tcc cgg ctc cga atc act atc gcc atc aac aac aca ctc        528
Pro Leu Pro Ser Arg Leu Arg Ile Thr Ile Ala Ile Asn Asn Thr Leu           
                165                 170                 175               

acc ccc acc acc ctg cca cca ggg acc atc caa tac ctg act gac acc        576
Thr Pro Thr Thr Leu Pro Pro Gly Thr Ile Gln Tyr Leu Thr Asp Thr           
            180                 185                 190                   

tcc aag tat ccc aag ggt tac ttt gtc cag aac aca tat ttt gac ttt        624
Ser Lys Tyr Pro Lys Gly Tyr Phe Val Gln Asn Thr Tyr Phe Asp Phe           
        195                 200                 205                       

ttc aac tac gct gga ctg cag cgg tct gta ctt ctg tac acg aca ccc        672
Phe Asn Tyr Ala Gly Leu Gln Arg Ser Val Leu Leu Tyr Thr Thr Pro           
    210                 215                 220                           

acc acc tac atc gat gac atc acc gtc acc acc agc gtg gag caa gac        720
Thr Thr Tyr Ile Asp Asp Ile Thr Val Thr Thr Ser Val Glu Gln Asp           
225                 230                 235                 240           

agt ggg ctg gtg aat tac cag atc tct gtc aag ggc agt aac ctg ttc        768
Ser Gly Leu Val Asn Tyr Gln Ile Ser Val Lys Gly Ser Asn Leu Phe           
                245                 250                 255               

aag ttg gaa gtg cgt ctt ttg gat gca gaa aac aaa gtc gtg gcg aat        816
Lys Leu Glu Val Arg Leu Leu Asp Ala Glu Asn Lys Val Val Ala Asn           
            260                 265                 270                   

ggg act ggg acc cag ggc caa ctt aag gtg cca ggt gtc agc ctc tgg        864
Gly Thr Gly Thr Gln Gly Gln Leu Lys Val Pro Gly Val Ser Leu Trp           
        275                 280                 285                       

tgg ccg tac ctg atg cac gaa cgc cct gcc tat ctg tat tca ttg gag        912
Trp Pro Tyr Leu Met His Glu Arg Pro Ala Tyr Leu Tyr Ser Leu Glu           
    290                 295                 300                           

gtg cag ctg act gca cag acg tca ctg ggg cct gtg tct gac ttc tac        960
Val Gln Leu Thr Ala Gln Thr Ser Leu Gly Pro Val Ser Asp Phe Tyr           
305                 310                 315                 320           

aca ctc cct gtg ggg atc cgc act gtg gct gtc acc aag agc cag ttc       1008
Thr Leu Pro Val Gly Ile Arg Thr Val Ala Val Thr Lys Ser Gln Phe           
                325                 330                 335               

ctc atc aat ggg aaa cct ttc tat ttc cac ggt gtc aac aag cat gag       1056
Leu Ile Asn Gly Lys Pro Phe Tyr Phe His Gly Val Asn Lys His Glu           
            340                 345                 350                   

gat gcg gac atc cga ggg aag ggc ttc gac tgg ccg ctg ctg gtg aag       1104
Asp Ala Asp Ile Arg Gly Lys Gly Phe Asp Trp Pro Leu Leu Val Lys           
        355                 360                 365                       

gac ttc aac ctg ctt cgc tgg ctt ggt gcc aac gct ttc cgt acc agc       1152
Asp Phe Asn Leu Leu Arg Trp Leu Gly Ala Asn Ala Phe Arg Thr Ser           
    370                 375                 380                           

cac tac ccc tat gca gag gaa gtg atg cag atg tgt gac cgc tat ggg       1200
His Tyr Pro Tyr Ala Glu Glu Val Met Gln Met Cys Asp Arg Tyr Gly           
385                 390                 395                 400           

att gtg gtc atc gat gag tgt ccc ggc gtg ggc ctg gcg ctg ccg cag       1248
Ile Val Val Ile Asp Glu Cys Pro Gly Val Gly Leu Ala Leu Pro Gln           
                405                 410                 415               

ttc ttc aac aac gtt tct ctg cat cac cac atg cag gtg atg gaa gaa       1296
Phe Phe Asn Asn Val Ser Leu His His His Met Gln Val Met Glu Glu           
            420                 425                 430                   

gtg gtg cgt agg gac aag aac cac ccc gcg gtc gtg atg tgg tct gtg       1344
Val Val Arg Arg Asp Lys Asn His Pro Ala Val Val Met Trp Ser Val           
        435                 440                 445                       

gcc aac gag cct gcg tcc cac cta gaa tct gct ggc tac tac ttg aag       1392
Ala Asn Glu Pro Ala Ser His Leu Glu Ser Ala Gly Tyr Tyr Leu Lys           
    450                 455                 460                           

atg gtg atc gct cac acc aaa tcc ttg gac ccc tcc cgg cct gtg acc       1440
Met Val Ile Ala His Thr Lys Ser Leu Asp Pro Ser Arg Pro Val Thr           
465                 470                 475                 480           

ttt gtg agc aac tct aac tat gca gca gac aag ggg gct ccg tat gtg       1488
Phe Val Ser Asn Ser Asn Tyr Ala Ala Asp Lys Gly Ala Pro Tyr Val           
                485                 490                 495               

gat gtg atc tgt ttg aac agc tac tac tct tgg tat cac gac tac ggg       1536
Asp Val Ile Cys Leu Asn Ser Tyr Tyr Ser Trp Tyr His Asp Tyr Gly           
            500                 505                 510                   

cac ctg gag ttg att cag ctg cag ctg gcc acc cag ttt gag aac tgg       1584
His Leu Glu Leu Ile Gln Leu Gln Leu Ala Thr Gln Phe Glu Asn Trp           
        515                 520                 525                       

tat aag aag tat cag aag ccc att att cag agc gag tat gga gca gaa       1632
Tyr Lys Lys Tyr Gln Lys Pro Ile Ile Gln Ser Glu Tyr Gly Ala Glu           
    530                 535                 540                           

acg att gca ggg ttt cac cag gat cca cct ctg atg ttc act gaa gag       1680
Thr Ile Ala Gly Phe His Gln Asp Pro Pro Leu Met Phe Thr Glu Glu           
545                 550                 555                 560           

tac cag aaa agt ctg cta gag cag tac cat ctg ggt ctg gat caa aaa       1728
Tyr Gln Lys Ser Leu Leu Glu Gln Tyr His Leu Gly Leu Asp Gln Lys           
                565                 570                 575               

cgc aga aaa tac gtg gtt gga gag ctc att tgg aat ttt gcc gat ttc       1776
Arg Arg Lys Tyr Val Val Gly Glu Leu Ile Trp Asn Phe Ala Asp Phe           
            580                 585                 590                   

atg act gaa cag tca ccg acg aga gtg ctg ggg aat aaa aag ggg atc       1824
Met Thr Glu Gln Ser Pro Thr Arg Val Leu Gly Asn Lys Lys Gly Ile           
        595                 600                 605                       

ttc act cgg cag aga caa cca aaa agt gca gcg ttc ctt ttg cga gag       1872
Phe Thr Arg Gln Arg Gln Pro Lys Ser Ala Ala Phe Leu Leu Arg Glu           
    610                 615                 620                           

aga tac tgg aag att gcc aat gaa acc agg tat ccc cac tca gta gcc       1920
Arg Tyr Trp Lys Ile Ala Asn Glu Thr Arg Tyr Pro His Ser Val Ala           
625                 630                 635                 640           

aag tca caa tgt ttg gaa aac agc ctg ttt act tga                       1956
Lys Ser Gln Cys Leu Glu Asn Ser Leu Phe Thr                               
                645                 650                                   


<210>  2
<211>  651
<212>  PRT
<213>  Homo sapiens beta-glucuronidase

<400>  2

Met Ala Arg Gly Ser Ala Val Ala Trp Ala Ala Leu Gly Pro Leu Leu 
1               5                   10                  15      


Trp Gly Cys Ala Leu Gly Leu Gln Gly Gly Met Leu Tyr Pro Gln Glu 
            20                  25                  30          


Ser Pro Ser Arg Glu Cys Lys Glu Leu Asp Gly Leu Trp Ser Phe Arg 
        35                  40                  45              


Ala Asp Phe Ser Asp Asn Arg Arg Arg Gly Phe Glu Glu Gln Trp Tyr 
    50                  55                  60                  


Arg Arg Pro Leu Trp Glu Ser Gly Pro Thr Val Asp Met Pro Val Pro 
65                  70                  75                  80  


Ser Ser Phe Asn Asp Ile Ser Gln Asp Trp Arg Leu Arg His Phe Val 
                85                  90                  95      


Gly Trp Val Trp Tyr Glu Arg Glu Val Ile Leu Pro Glu Arg Trp Thr 
            100                 105                 110         


Gln Asp Leu Arg Thr Arg Val Val Leu Arg Ile Gly Ser Ala His Ser 
        115                 120                 125             


Tyr Ala Ile Val Trp Val Asn Gly Val Asp Thr Leu Glu His Glu Gly 
    130                 135                 140                 


Gly Tyr Leu Pro Phe Glu Ala Asp Ile Ser Asn Leu Val Gln Val Gly 
145                 150                 155                 160 


Pro Leu Pro Ser Arg Leu Arg Ile Thr Ile Ala Ile Asn Asn Thr Leu 
                165                 170                 175     


Thr Pro Thr Thr Leu Pro Pro Gly Thr Ile Gln Tyr Leu Thr Asp Thr 
            180                 185                 190         


Ser Lys Tyr Pro Lys Gly Tyr Phe Val Gln Asn Thr Tyr Phe Asp Phe 
        195                 200                 205             


Phe Asn Tyr Ala Gly Leu Gln Arg Ser Val Leu Leu Tyr Thr Thr Pro 
    210                 215                 220                 


Thr Thr Tyr Ile Asp Asp Ile Thr Val Thr Thr Ser Val Glu Gln Asp 
225                 230                 235                 240 


Ser Gly Leu Val Asn Tyr Gln Ile Ser Val Lys Gly Ser Asn Leu Phe 
                245                 250                 255     


Lys Leu Glu Val Arg Leu Leu Asp Ala Glu Asn Lys Val Val Ala Asn 
            260                 265                 270         


Gly Thr Gly Thr Gln Gly Gln Leu Lys Val Pro Gly Val Ser Leu Trp 
        275                 280                 285             


Trp Pro Tyr Leu Met His Glu Arg Pro Ala Tyr Leu Tyr Ser Leu Glu 
    290                 295                 300                 


Val Gln Leu Thr Ala Gln Thr Ser Leu Gly Pro Val Ser Asp Phe Tyr 
305                 310                 315                 320 


Thr Leu Pro Val Gly Ile Arg Thr Val Ala Val Thr Lys Ser Gln Phe 
                325                 330                 335     


Leu Ile Asn Gly Lys Pro Phe Tyr Phe His Gly Val Asn Lys His Glu 
            340                 345                 350         


Asp Ala Asp Ile Arg Gly Lys Gly Phe Asp Trp Pro Leu Leu Val Lys 
        355                 360                 365             


Asp Phe Asn Leu Leu Arg Trp Leu Gly Ala Asn Ala Phe Arg Thr Ser 
    370                 375                 380                 


His Tyr Pro Tyr Ala Glu Glu Val Met Gln Met Cys Asp Arg Tyr Gly 
385                 390                 395                 400 


Ile Val Val Ile Asp Glu Cys Pro Gly Val Gly Leu Ala Leu Pro Gln 
                405                 410                 415     


Phe Phe Asn Asn Val Ser Leu His His His Met Gln Val Met Glu Glu 
            420                 425                 430         


Val Val Arg Arg Asp Lys Asn His Pro Ala Val Val Met Trp Ser Val 
        435                 440                 445             


Ala Asn Glu Pro Ala Ser His Leu Glu Ser Ala Gly Tyr Tyr Leu Lys 
    450                 455                 460                 


Met Val Ile Ala His Thr Lys Ser Leu Asp Pro Ser Arg Pro Val Thr 
465                 470                 475                 480 


Phe Val Ser Asn Ser Asn Tyr Ala Ala Asp Lys Gly Ala Pro Tyr Val 
                485                 490                 495     


Asp Val Ile Cys Leu Asn Ser Tyr Tyr Ser Trp Tyr His Asp Tyr Gly 
            500                 505                 510         


His Leu Glu Leu Ile Gln Leu Gln Leu Ala Thr Gln Phe Glu Asn Trp 
        515                 520                 525             


Tyr Lys Lys Tyr Gln Lys Pro Ile Ile Gln Ser Glu Tyr Gly Ala Glu 
    530                 535                 540                 


Thr Ile Ala Gly Phe His Gln Asp Pro Pro Leu Met Phe Thr Glu Glu 
545                 550                 555                 560 


Tyr Gln Lys Ser Leu Leu Glu Gln Tyr His Leu Gly Leu Asp Gln Lys 
                565                 570                 575     


Arg Arg Lys Tyr Val Val Gly Glu Leu Ile Trp Asn Phe Ala Asp Phe 
            580                 585                 590         


Met Thr Glu Gln Ser Pro Thr Arg Val Leu Gly Asn Lys Lys Gly Ile 
        595                 600                 605             


Phe Thr Arg Gln Arg Gln Pro Lys Ser Ala Ala Phe Leu Leu Arg Glu 
    610                 615                 620                 


Arg Tyr Trp Lys Ile Ala Asn Glu Thr Arg Tyr Pro His Ser Val Ala 
625                 630                 635                 640 


Lys Ser Gln Cys Leu Glu Asn Ser Leu Phe Thr 
                645                 650     


<210>  3
<211>  1962
<212>  DNA
<213>  Homo sapiens alpha-iduronidase


<220>
<221>  CDS
<222>  (1)..(1962)

<400>  3
atg cgt ccc ctg cgc ccc cgc gcc gcg ctg ctg gcg ctc ctg gcc tcg         48
Met Arg Pro Leu Arg Pro Arg Ala Ala Leu Leu Ala Leu Leu Ala Ser           
1               5                   10                  15                

ctc ctg gcc gcg ccc ccg gtg gcc ccg gcc gag gcc ccg cac ctg gtg         96
Leu Leu Ala Ala Pro Pro Val Ala Pro Ala Glu Ala Pro His Leu Val           
            20                  25                  30                    

cat gtg gac gcg gcc cgc gcg ctg tgg ccc ctg cgg cgc ttc tgg agg        144
His Val Asp Ala Ala Arg Ala Leu Trp Pro Leu Arg Arg Phe Trp Arg           
        35                  40                  45                        

agc aca ggc ttc tgc ccc ccg ctg cca cac agc cag gct gac cag tac        192
Ser Thr Gly Phe Cys Pro Pro Leu Pro His Ser Gln Ala Asp Gln Tyr           
    50                  55                  60                            

gtc ctc agc tgg gac cag cag ctc aac ctc gcc tat gtg ggc gcc gtc        240
Val Leu Ser Trp Asp Gln Gln Leu Asn Leu Ala Tyr Val Gly Ala Val           
65                  70                  75                  80            

cct cac cgc ggc atc aag cag gtc cgg acc cac tgg ctg ctg gag ctt        288
Pro His Arg Gly Ile Lys Gln Val Arg Thr His Trp Leu Leu Glu Leu           
                85                  90                  95                

gtc acc acc agg ggg tcc act gga cgg ggc ctg agc tac aac ttc acc        336
Val Thr Thr Arg Gly Ser Thr Gly Arg Gly Leu Ser Tyr Asn Phe Thr           
            100                 105                 110                   

cac ctg gac ggg tac ctg gac ctt ctc agg gag aac cag ctc ctc cca        384
His Leu Asp Gly Tyr Leu Asp Leu Leu Arg Glu Asn Gln Leu Leu Pro           
        115                 120                 125                       

ggg ttt gag ctg atg ggc agc gcc tcg ggc cac ttc act gac ttt gag        432
Gly Phe Glu Leu Met Gly Ser Ala Ser Gly His Phe Thr Asp Phe Glu           
    130                 135                 140                           

gac aag cag cag gtg ttt gag tgg aag gac ttg gtc tcc agc ctg gcc        480
Asp Lys Gln Gln Val Phe Glu Trp Lys Asp Leu Val Ser Ser Leu Ala           
145                 150                 155                 160           

agg aga tac atc ggt agg tac gga ctg gcg cat gtt tcc aag tgg aac        528
Arg Arg Tyr Ile Gly Arg Tyr Gly Leu Ala His Val Ser Lys Trp Asn           
                165                 170                 175               

ttc gag acg tgg aat gag cca gac cac cac gac ttt gac aac gtc tcc        576
Phe Glu Thr Trp Asn Glu Pro Asp His His Asp Phe Asp Asn Val Ser           
            180                 185                 190                   

atg acc atg caa ggc ttc ctg aac tac tac gat gcc tgc tcg gag ggt        624
Met Thr Met Gln Gly Phe Leu Asn Tyr Tyr Asp Ala Cys Ser Glu Gly           
        195                 200                 205                       

ctg cgc gcc gcc agc ccc gcc ctg cgg ctg gga ggc ccc ggc gac tcc        672
Leu Arg Ala Ala Ser Pro Ala Leu Arg Leu Gly Gly Pro Gly Asp Ser           
    210                 215                 220                           

ttc cac acc cca ccg cga tcc ccg ctg agc tgg ggc ctc ctg cgc cac        720
Phe His Thr Pro Pro Arg Ser Pro Leu Ser Trp Gly Leu Leu Arg His           
225                 230                 235                 240           

tgc cac gac ggt acc aac ttc ttc act ggg gag gcg ggc gtg cgg ctg        768
Cys His Asp Gly Thr Asn Phe Phe Thr Gly Glu Ala Gly Val Arg Leu           
                245                 250                 255               

gac tac atc tcc ctc cac agg aag ggt gcg cgc agc tcc atc tcc atc        816
Asp Tyr Ile Ser Leu His Arg Lys Gly Ala Arg Ser Ser Ile Ser Ile           
            260                 265                 270                   

ctg gag cag gag aag gtc gtc gcg cag cag atc cgg cag ctc ttc ccc        864
Leu Glu Gln Glu Lys Val Val Ala Gln Gln Ile Arg Gln Leu Phe Pro           
        275                 280                 285                       

aag ttc gcg gac acc ccc att tac aac gac gag gcg gac ccg ctg gtg        912
Lys Phe Ala Asp Thr Pro Ile Tyr Asn Asp Glu Ala Asp Pro Leu Val           
    290                 295                 300                           

ggc tgg tcc ctg cca cag ccg tgg agg gcg gac gtg acc tac gcg gcc        960
Gly Trp Ser Leu Pro Gln Pro Trp Arg Ala Asp Val Thr Tyr Ala Ala           
305                 310                 315                 320           

atg gtg gtg aag gtc atc gcg cag cat cag aac ctg cta ctg gcc aac       1008
Met Val Val Lys Val Ile Ala Gln His Gln Asn Leu Leu Leu Ala Asn           
                325                 330                 335               

acc acc tcc gcc ttc ccc tac gcg ctc ctg agc aac gac aat gcc ttc       1056
Thr Thr Ser Ala Phe Pro Tyr Ala Leu Leu Ser Asn Asp Asn Ala Phe           
            340                 345                 350                   

ctg agc tac cac ccg cac ccc ttc gcg cag cgc acg ctc acc gcg cgc       1104
Leu Ser Tyr His Pro His Pro Phe Ala Gln Arg Thr Leu Thr Ala Arg           
        355                 360                 365                       

ttc cag gtc aac aac acc cgc ccg ccg cac gtg cag ctg ttg cgc aag       1152
Phe Gln Val Asn Asn Thr Arg Pro Pro His Val Gln Leu Leu Arg Lys           
    370                 375                 380                           

ccg gtg ctc acg gcc atg ggg ctg ctg gcg ctg ctg gat gag gag cag       1200
Pro Val Leu Thr Ala Met Gly Leu Leu Ala Leu Leu Asp Glu Glu Gln           
385                 390                 395                 400           

ctc tgg gcc gaa gtg tcg cag gcc ggg acc gtc ctg gac agc aac cac       1248
Leu Trp Ala Glu Val Ser Gln Ala Gly Thr Val Leu Asp Ser Asn His           
                405                 410                 415               

acg gtg ggc gtc ctg gcc agc gcc cac cgc ccc cag ggc ccg gcc gac       1296
Thr Val Gly Val Leu Ala Ser Ala His Arg Pro Gln Gly Pro Ala Asp           
            420                 425                 430                   

gcc tgg cgc gcc gcg gtg ctg atc tac gcg agc gac gac acc cgc gcc       1344
Ala Trp Arg Ala Ala Val Leu Ile Tyr Ala Ser Asp Asp Thr Arg Ala           
        435                 440                 445                       

cac ccc aac cgc agc gtc gcg gtg acc ctg cgg ctg cgc ggg gtg ccc       1392
His Pro Asn Arg Ser Val Ala Val Thr Leu Arg Leu Arg Gly Val Pro           
    450                 455                 460                           

ccc ggc ccg ggc ctg gtc tac gtc acg cgc tac ctg gac aac ggg ctc       1440
Pro Gly Pro Gly Leu Val Tyr Val Thr Arg Tyr Leu Asp Asn Gly Leu           
465                 470                 475                 480           

tgc agc ccc gac ggc gag tgg cgg cgc ctg ggc cgg ccc gtc ttc ccc       1488
Cys Ser Pro Asp Gly Glu Trp Arg Arg Leu Gly Arg Pro Val Phe Pro           
                485                 490                 495               

acg gca gag cag ttc cgg cgc atg cgc gcg gct gag gac ccg gtg gcc       1536
Thr Ala Glu Gln Phe Arg Arg Met Arg Ala Ala Glu Asp Pro Val Ala           
            500                 505                 510                   

gcg gcg ccc cgc ccc tta ccc gcc ggc ggc cgc ctg acc ctg cgc ccc       1584
Ala Ala Pro Arg Pro Leu Pro Ala Gly Gly Arg Leu Thr Leu Arg Pro           
        515                 520                 525                       

gcg ctg cgg ctg ccg tcg ctt ttg ctg gtg cac gtg tgt gcg cgc ccc       1632
Ala Leu Arg Leu Pro Ser Leu Leu Leu Val His Val Cys Ala Arg Pro           
    530                 535                 540                           

gag aag ccg ccc ggg cag gtc acg cgg ctc cgc gcc ctg ccc ctg acc       1680
Glu Lys Pro Pro Gly Gln Val Thr Arg Leu Arg Ala Leu Pro Leu Thr           
545                 550                 555                 560           

caa ggg cag ctg gtt ctg gtc tgg tcg gat gaa cac gtg ggc tcc aag       1728
Gln Gly Gln Leu Val Leu Val Trp Ser Asp Glu His Val Gly Ser Lys           
                565                 570                 575               

tgc ctg tgg aca tac gag atc cag ttc tct cag gac ggt aag gcg tac       1776
Cys Leu Trp Thr Tyr Glu Ile Gln Phe Ser Gln Asp Gly Lys Ala Tyr           
            580                 585                 590                   

acc ccg gtc agc agg aag cca tcg acc ttc aac ctc ttt gtg ttc agc       1824
Thr Pro Val Ser Arg Lys Pro Ser Thr Phe Asn Leu Phe Val Phe Ser           
        595                 600                 605                       

cca gac aca ggt gct gtc tct ggc tcc tac cga gtt cga gcc ctg gac       1872
Pro Asp Thr Gly Ala Val Ser Gly Ser Tyr Arg Val Arg Ala Leu Asp           
    610                 615                 620                           

tac tgg gcc cga cca ggc ccc ttc tcg gac cct gtg ccg tac ctg gag       1920
Tyr Trp Ala Arg Pro Gly Pro Phe Ser Asp Pro Val Pro Tyr Leu Glu           
625                 630                 635                 640           

gtc cct gtg cca aga ggg ccc cca tcc ccg ggc aat cca tga               1962
Val Pro Val Pro Arg Gly Pro Pro Ser Pro Gly Asn Pro                       
                645                 650                                   


<210>  4
<211>  653
<212>  PRT
<213>  Homo sapiens alpha-iduronidase

<400>  4

Met Arg Pro Leu Arg Pro Arg Ala Ala Leu Leu Ala Leu Leu Ala Ser 
1               5                   10                  15      


Leu Leu Ala Ala Pro Pro Val Ala Pro Ala Glu Ala Pro His Leu Val 
            20                  25                  30          


His Val Asp Ala Ala Arg Ala Leu Trp Pro Leu Arg Arg Phe Trp Arg 
        35                  40                  45              


Ser Thr Gly Phe Cys Pro Pro Leu Pro His Ser Gln Ala Asp Gln Tyr 
    50                  55                  60                  


Val Leu Ser Trp Asp Gln Gln Leu Asn Leu Ala Tyr Val Gly Ala Val 
65                  70                  75                  80  


Pro His Arg Gly Ile Lys Gln Val Arg Thr His Trp Leu Leu Glu Leu 
                85                  90                  95      


Val Thr Thr Arg Gly Ser Thr Gly Arg Gly Leu Ser Tyr Asn Phe Thr 
            100                 105                 110         


His Leu Asp Gly Tyr Leu Asp Leu Leu Arg Glu Asn Gln Leu Leu Pro 
        115                 120                 125             


Gly Phe Glu Leu Met Gly Ser Ala Ser Gly His Phe Thr Asp Phe Glu 
    130                 135                 140                 


Asp Lys Gln Gln Val Phe Glu Trp Lys Asp Leu Val Ser Ser Leu Ala 
145                 150                 155                 160 


Arg Arg Tyr Ile Gly Arg Tyr Gly Leu Ala His Val Ser Lys Trp Asn 
                165                 170                 175     


Phe Glu Thr Trp Asn Glu Pro Asp His His Asp Phe Asp Asn Val Ser 
            180                 185                 190         


Met Thr Met Gln Gly Phe Leu Asn Tyr Tyr Asp Ala Cys Ser Glu Gly 
        195                 200                 205             


Leu Arg Ala Ala Ser Pro Ala Leu Arg Leu Gly Gly Pro Gly Asp Ser 
    210                 215                 220                 


Phe His Thr Pro Pro Arg Ser Pro Leu Ser Trp Gly Leu Leu Arg His 
225                 230                 235                 240 


Cys His Asp Gly Thr Asn Phe Phe Thr Gly Glu Ala Gly Val Arg Leu 
                245                 250                 255     


Asp Tyr Ile Ser Leu His Arg Lys Gly Ala Arg Ser Ser Ile Ser Ile 
            260                 265                 270         


Leu Glu Gln Glu Lys Val Val Ala Gln Gln Ile Arg Gln Leu Phe Pro 
        275                 280                 285             


Lys Phe Ala Asp Thr Pro Ile Tyr Asn Asp Glu Ala Asp Pro Leu Val 
    290                 295                 300                 


Gly Trp Ser Leu Pro Gln Pro Trp Arg Ala Asp Val Thr Tyr Ala Ala 
305                 310                 315                 320 


Met Val Val Lys Val Ile Ala Gln His Gln Asn Leu Leu Leu Ala Asn 
                325                 330                 335     


Thr Thr Ser Ala Phe Pro Tyr Ala Leu Leu Ser Asn Asp Asn Ala Phe 
            340                 345                 350         


Leu Ser Tyr His Pro His Pro Phe Ala Gln Arg Thr Leu Thr Ala Arg 
        355                 360                 365             


Phe Gln Val Asn Asn Thr Arg Pro Pro His Val Gln Leu Leu Arg Lys 
    370                 375                 380                 


Pro Val Leu Thr Ala Met Gly Leu Leu Ala Leu Leu Asp Glu Glu Gln 
385                 390                 395                 400 


Leu Trp Ala Glu Val Ser Gln Ala Gly Thr Val Leu Asp Ser Asn His 
                405                 410                 415     


Thr Val Gly Val Leu Ala Ser Ala His Arg Pro Gln Gly Pro Ala Asp 
            420                 425                 430         


Ala Trp Arg Ala Ala Val Leu Ile Tyr Ala Ser Asp Asp Thr Arg Ala 
        435                 440                 445             


His Pro Asn Arg Ser Val Ala Val Thr Leu Arg Leu Arg Gly Val Pro 
    450                 455                 460                 


Pro Gly Pro Gly Leu Val Tyr Val Thr Arg Tyr Leu Asp Asn Gly Leu 
465                 470                 475                 480 


Cys Ser Pro Asp Gly Glu Trp Arg Arg Leu Gly Arg Pro Val Phe Pro 
                485                 490                 495     


Thr Ala Glu Gln Phe Arg Arg Met Arg Ala Ala Glu Asp Pro Val Ala 
            500                 505                 510         


Ala Ala Pro Arg Pro Leu Pro Ala Gly Gly Arg Leu Thr Leu Arg Pro 
        515                 520                 525             


Ala Leu Arg Leu Pro Ser Leu Leu Leu Val His Val Cys Ala Arg Pro 
    530                 535                 540                 


Glu Lys Pro Pro Gly Gln Val Thr Arg Leu Arg Ala Leu Pro Leu Thr 
545                 550                 555                 560 


Gln Gly Gln Leu Val Leu Val Trp Ser Asp Glu His Val Gly Ser Lys 
                565                 570                 575     


Cys Leu Trp Thr Tyr Glu Ile Gln Phe Ser Gln Asp Gly Lys Ala Tyr 
            580                 585                 590         


Thr Pro Val Ser Arg Lys Pro Ser Thr Phe Asn Leu Phe Val Phe Ser 
        595                 600                 605             


Pro Asp Thr Gly Ala Val Ser Gly Ser Tyr Arg Val Arg Ala Leu Asp 
    610                 615                 620                 


Tyr Trp Ala Arg Pro Gly Pro Phe Ser Asp Pro Val Pro Tyr Leu Glu 
625                 630                 635                 640 


Val Pro Val Pro Arg Gly Pro Pro Ser Pro Gly Asn Pro 
                645                 650             


