                         SEQUENCE LISTING

<110>  AMICUS THERAPEUTICS
       GOTSCHALL, Russell
       DO, Hung
 
<120>  HIGHLY POTENT MODIFIED ACID ALPHA-GLUCOSIDASE WITH ENHANCED CARBOHYDRATES

<130>  442420WO

<150>  US 62/135,345
<151>  2015-03-19

<150>  US 62/112,643
<151>  2015-02-05

<150>  US 62/057,847
<151>  2014-09-30

<150>  US 62/057,842
<151>  2014-09-30

<160>  4     

<170>  PatentIn version 3.5

<210>  1
<211>  952
<212>  PRT
<213>  Homo sapiens


<220>
<221>  MISC_FEATURE
<222>  (1)..(952)
<223>  Sequence 4 from patent US 8592362GenBank: AHE24104.1

<400>  1

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


Ala Leu Val Ser Leu Ala Thr Ala Ala Leu Leu Gly His Ile Leu Leu 
            20                  25                  30          


His Asp Phe Leu Leu Val Pro Arg Glu Leu Ser Gly Ser Ser Pro Val 
        35                  40                  45              


Leu Glu Glu Thr His Pro Ala His Gln Gln Gly Ala Ser Arg Pro Gly 
    50                  55                  60                  


Pro Arg Asp Ala Gln Ala His Pro Gly Arg Pro Arg Ala Val Pro Thr 
65                  70                  75                  80  


Gln Cys Asp Val Pro Pro Asn Ser Arg Phe Asp Cys Ala Pro Asp Lys 
                85                  90                  95      


Ala Ile Thr Gln Glu Gln Cys Glu Ala Arg Gly Cys Cys Tyr Ile Pro 
            100                 105                 110         


Ala Lys Gln Gly Leu Gln Gly Ala Gln Met Gly Gln Pro Trp Cys Phe 
        115                 120                 125             


Phe Pro Pro Ser Tyr Pro Ser Tyr Lys Leu Glu Asn Leu Ser Ser Ser 
    130                 135                 140                 


Glu Met Gly Tyr Thr Ala Thr Leu Thr Arg Thr Thr Pro Thr Phe Phe 
145                 150                 155                 160 


Pro Lys Asp Ile Leu Thr Leu Arg Leu Asp Val Met Met Glu Thr Glu 
                165                 170                 175     


Asn Arg Leu His Phe Thr Ile Lys Asp Pro Ala Asn Arg Arg Tyr Glu 
            180                 185                 190         


Val Pro Leu Glu Thr Pro Arg Val His Ser Arg Ala Pro Ser Pro Leu 
        195                 200                 205             


Tyr Ser Val Glu Phe Ser Glu Glu Pro Phe Gly Val Ile Val His Arg 
    210                 215                 220                 


Gln Leu Asp Gly Arg Val Leu Leu Asn Thr Thr Val Ala Pro Leu Phe 
225                 230                 235                 240 


Phe Ala Asp Gln Phe Leu Gln Leu Ser Thr Ser Leu Pro Ser Gln Tyr 
                245                 250                 255     


Ile Thr Gly Leu Ala Glu His Leu Ser Pro Leu Met Leu Ser Thr Ser 
            260                 265                 270         


Trp Thr Arg Ile Thr Leu Trp Asn Arg Asp Leu Ala Pro Thr Pro Gly 
        275                 280                 285             


Ala Asn Leu Tyr Gly Ser His Pro Phe Tyr Leu Ala Leu Glu Asp Gly 
    290                 295                 300                 


Gly Ser Ala His Gly Val Phe Leu Leu Asn Ser Asn Ala Met Asp Val 
305                 310                 315                 320 


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


Leu Asp Val Tyr Ile Phe Leu Gly Pro Glu Pro Lys Ser Val Val Gln 
            340                 345                 350         


Gln Tyr Leu Asp Val Val Gly Tyr Pro Phe Met Pro Pro Tyr Trp Gly 
        355                 360                 365             


Leu Gly Phe His Leu Cys Arg Trp Gly Tyr Ser Ser Thr Ala Ile Thr 
    370                 375                 380                 


Arg Gln Val Val Glu Asn Met Thr Arg Ala His Phe Pro Leu Asp Val 
385                 390                 395                 400 


Gln Trp Asn Asp Leu Asp Tyr Met Asp Ser Arg Arg Asp Phe Thr Phe 
                405                 410                 415     


Asn Lys Asp Gly Phe Arg Asp Phe Pro Ala Met Val Gln Glu Leu His 
            420                 425                 430         


Gln Gly Gly Arg Arg Tyr Met Met Ile Val Asp Pro Ala Ile Ser Ser 
        435                 440                 445             


Ser Gly Pro Ala Gly Ser Tyr Arg Pro Tyr Asp Glu Gly Leu Arg Arg 
    450                 455                 460                 


Gly Val Phe Ile Thr Asn Glu Thr Gly Gln Pro Leu Ile Gly Lys Val 
465                 470                 475                 480 


Trp Pro Gly Ser Thr Ala Phe Pro Asp Phe Thr Asn Pro Thr Ala Leu 
                485                 490                 495     


Ala Trp Trp Glu Asp Met Val Ala Glu Phe His Asp Gln Val Pro Phe 
            500                 505                 510         


Asp Gly Met Trp Ile Asp Met Asn Glu Pro Ser Asn Phe Ile Arg Gly 
        515                 520                 525             


Ser Glu Asp Gly Cys Pro Asn Asn Glu Leu Glu Asn Pro Pro Tyr Val 
    530                 535                 540                 


Pro Gly Val Val Gly Gly Thr Leu Gln Ala Ala Thr Ile Cys Ala Ser 
545                 550                 555                 560 


Ser His Gln Phe Leu Ser Thr His Tyr Asn Leu His Asn Leu Tyr Gly 
                565                 570                 575     


Leu Thr Glu Ala Ile Ala Ser His Arg Ala Leu Val Lys Ala Arg Gly 
            580                 585                 590         


Thr Arg Pro Phe Val Ile Ser Arg Ser Thr Phe Ala Gly His Gly Arg 
        595                 600                 605             


Tyr Ala Gly His Trp Thr Gly Asp Val Trp Ser Ser Trp Glu Gln Leu 
    610                 615                 620                 


Ala Ser Ser Val Pro Glu Ile Leu Gln Phe Asn Leu Leu Gly Val Pro 
625                 630                 635                 640 


Leu Val Gly Ala Asp Val Cys Gly Phe Leu Gly Asn Thr Ser Glu Glu 
                645                 650                 655     


Leu Cys Val Arg Trp Thr Gln Leu Gly Ala Phe Tyr Pro Phe Met Arg 
            660                 665                 670         


Asn His Asn Ser Leu Leu Ser Leu Pro Gln Glu Pro Tyr Ser Phe Ser 
        675                 680                 685             


Glu Pro Ala Gln Gln Ala Met Arg Lys Ala Leu Thr Leu Arg Tyr Ala 
    690                 695                 700                 


Leu Leu Pro His Leu Tyr Thr Leu Phe His Gln Ala His Val Ala Gly 
705                 710                 715                 720 


Glu Thr Val Ala Arg Pro Leu Phe Leu Glu Phe Pro Lys Asp Ser Ser 
                725                 730                 735     


Thr Trp Thr Val Asp His Gln Leu Leu Trp Gly Glu Ala Leu Leu Ile 
            740                 745                 750         


Thr Pro Val Leu Gln Ala Gly Lys Ala Glu Val Thr Gly Tyr Phe Pro 
        755                 760                 765             


Leu Gly Thr Trp Tyr Asp Leu Gln Thr Val Pro Ile Glu Ala Leu Gly 
    770                 775                 780                 


Ser Leu Pro Pro Pro Pro Ala Ala Pro Arg Glu Pro Ala Ile His Ser 
785                 790                 795                 800 


Glu Gly Gln Trp Val Thr Leu Pro Ala Pro Leu Asp Thr Ile Asn Val 
                805                 810                 815     


His Leu Arg Ala Gly Tyr Ile Ile Pro Leu Gln Gly Pro Gly Leu Thr 
            820                 825                 830         


Thr Thr Glu Ser Arg Gln Gln Pro Met Ala Leu Ala Val Ala Leu Thr 
        835                 840                 845             


Lys Gly Gly Glu Ala Arg Gly Glu Leu Phe Trp Asp Asp Gly Glu Ser 
    850                 855                 860                 


Leu Glu Val Leu Glu Arg Gly Ala Tyr Thr Gln Val Ile Phe Leu Ala 
865                 870                 875                 880 


Arg Asn Asn Thr Ile Val Asn Glu Leu Val Arg Val Thr Ser Glu Gly 
                885                 890                 895     


Ala Gly Leu Gln Leu Gln Lys Val Thr Val Leu Gly Val Ala Thr Ala 
            900                 905                 910         


Pro Gln Gln Val Leu Ser Asn Gly Val Pro Val Ser Asn Phe Thr Tyr 
        915                 920                 925             


Ser Pro Asp Thr Lys Val Leu Asp Ile Cys Val Ser Leu Leu Met Gly 
    930                 935                 940                 


Glu Gln Phe Leu Val Ser Trp Cys 
945                 950         


<210>  2
<211>  3624
<212>  DNA
<213>  Homo sapiens


<220>
<221>  CDS
<222>  (220)..(3078)
<223>  H.sapiens GAA mRNA for lysosomal alpha-glucosidase (acid        maltase);  GenBank: Y00839.1

<400>  2
cagttgggaa agctgaggtt gtcgccgggg ccgcgggtgg aggtcgggga tgaggcagca       60

ggtaggacag tgacctcggt gacgcgaagg accccggcca cctctaggtt ctcctcgtcc      120

gcccgttgtt cagcgaggga ggctctgggc ctgccgcagc tgacggggaa actgaggcac      180

ggagcgggcc tgtaggagct gtccaggcca tctccaacc atg gga gtg agg cac         234
                                           Met Gly Val Arg His            
                                           1               5              

ccg ccc tgc tcc cac cgg ctc ctg gcc gtc tgc gcc ctc gtg tcc ttg        282
Pro Pro Cys Ser His Arg Leu Leu Ala Val Cys Ala Leu Val Ser Leu           
                10                  15                  20                

gca acc gct gca ctc ctg ggg cac atc cta ctc cat gat ttc ctg ctg        330
Ala Thr Ala Ala Leu Leu Gly His Ile Leu Leu His Asp Phe Leu Leu           
            25                  30                  35                    

gtt ccc cga gag ctg agt ggc tcc tcc cca gtc ctg gag gag act cac        378
Val Pro Arg Glu Leu Ser Gly Ser Ser Pro Val Leu Glu Glu Thr His           
        40                  45                  50                        

cca gct cac cag cag gga gcc agc aga cca ggg ccc cgg gat gcc cag        426
Pro Ala His Gln Gln Gly Ala Ser Arg Pro Gly Pro Arg Asp Ala Gln           
    55                  60                  65                            

gca cac ccc ggc cgt ccc aga gca gtg ccc aca cag tgc gac gtc ccc        474
Ala His Pro Gly Arg Pro Arg Ala Val Pro Thr Gln Cys Asp Val Pro           
70                  75                  80                  85            

ccc aac agc cgc ttc gat tgc gcc cct gac aag gcc atc acc cag gaa        522
Pro Asn Ser Arg Phe Asp Cys Ala Pro Asp Lys Ala Ile Thr Gln Glu           
                90                  95                  100               

cag tgc gag gcc cgc ggc tgc tgc tac atc cct gca aag cag ggg ctg        570
Gln Cys Glu Ala Arg Gly Cys Cys Tyr Ile Pro Ala Lys Gln Gly Leu           
            105                 110                 115                   

cag gga gcc cag atg ggg cag ccc tgg tgc ttc ttc cca ccc agc tac        618
Gln Gly Ala Gln Met Gly Gln Pro Trp Cys Phe Phe Pro Pro Ser Tyr           
        120                 125                 130                       

ccc agc tac aag ctg gag aac ctg agc tcc tct gaa atg ggc tac acg        666
Pro Ser Tyr Lys Leu Glu Asn Leu Ser Ser Ser Glu Met Gly Tyr Thr           
    135                 140                 145                           

gcc acc ctg acc cgt acc acc ccc acc ttc ttc ccc aag gac atc ctg        714
Ala Thr Leu Thr Arg Thr Thr Pro Thr Phe Phe Pro Lys Asp Ile Leu           
150                 155                 160                 165           

acc ctg cgg ctg gac gtg atg atg gag act gag aac cgc ctc cac ttc        762
Thr Leu Arg Leu Asp Val Met Met Glu Thr Glu Asn Arg Leu His Phe           
                170                 175                 180               

acg atc aaa gat cca gct aac agg cgc tac gag gtg ccc ttg gag acc        810
Thr Ile Lys Asp Pro Ala Asn Arg Arg Tyr Glu Val Pro Leu Glu Thr           
            185                 190                 195                   

ccg cgt gtc cac agc cgg gca ccg tcc cca ctc tac agc gtg gag ttc        858
Pro Arg Val His Ser Arg Ala Pro Ser Pro Leu Tyr Ser Val Glu Phe           
        200                 205                 210                       

tcc gag gag ccc ttc ggg gtg atc gtg cac cgg cag ctg gac ggc cgc        906
Ser Glu Glu Pro Phe Gly Val Ile Val His Arg Gln Leu Asp Gly Arg           
    215                 220                 225                           

gtg ctg ctg aac acg acg gtg gcg ccc ctg ttc ttt gcg gac cag ttc        954
Val Leu Leu Asn Thr Thr Val Ala Pro Leu Phe Phe Ala Asp Gln Phe           
230                 235                 240                 245           

ctt cag ctg tcc acc tcg ctg ccc tcg cag tat atc aca ggc ctc gcc       1002
Leu Gln Leu Ser Thr Ser Leu Pro Ser Gln Tyr Ile Thr Gly Leu Ala           
                250                 255                 260               

gag cac ctc agt ccc ctg atg ctc agc acc agc tgg acc agg atc acc       1050
Glu His Leu Ser Pro Leu Met Leu Ser Thr Ser Trp Thr Arg Ile Thr           
            265                 270                 275                   

ctg tgg aac cgg gac ctt gcg ccc acg ccc ggt gcg aac ctc tac ggg       1098
Leu Trp Asn Arg Asp Leu Ala Pro Thr Pro Gly Ala Asn Leu Tyr Gly           
        280                 285                 290                       

tct cac cct ttc tac ctg gcg ctg gag gac ggc ggg tcg gca cac ggg       1146
Ser His Pro Phe Tyr Leu Ala Leu Glu Asp Gly Gly Ser Ala His Gly           
    295                 300                 305                           

gtg ttc ctg cta aac agc aat gcc atg gat gtg gtc ctg cag ccg agc       1194
Val Phe Leu Leu Asn Ser Asn Ala Met Asp Val Val Leu Gln Pro Ser           
310                 315                 320                 325           

cct gcc ctt agc tgg agg tcg aca ggt ggg atc ctg gat gtc tac atc       1242
Pro Ala Leu Ser Trp Arg Ser Thr Gly Gly Ile Leu Asp Val Tyr Ile           
                330                 335                 340               

ttc ctg ggc cca gag ccc aag agc gtg gtg cag cag tac ctg gac gtt       1290
Phe Leu Gly Pro Glu Pro Lys Ser Val Val Gln Gln Tyr Leu Asp Val           
            345                 350                 355                   

gtg gga tac ccg ttc atg ccg cca tac tgg ggc ctg ggc ttc cac ctg       1338
Val Gly Tyr Pro Phe Met Pro Pro Tyr Trp Gly Leu Gly Phe His Leu           
        360                 365                 370                       

tgc cgc tgg ggc tac tcc tcc acc gct atc acc cgc cag gtg gtg gag       1386
Cys Arg Trp Gly Tyr Ser Ser Thr Ala Ile Thr Arg Gln Val Val Glu           
    375                 380                 385                           

aac atg acc agg gcc cac ttc ccc ctg gac gtc caa tgg aac gac ctg       1434
Asn Met Thr Arg Ala His Phe Pro Leu Asp Val Gln Trp Asn Asp Leu           
390                 395                 400                 405           

gac tac atg gac tcc cgg agg gac ttc acg ttc aac aag gat ggc ttc       1482
Asp Tyr Met Asp Ser Arg Arg Asp Phe Thr Phe Asn Lys Asp Gly Phe           
                410                 415                 420               

cgg gac ttc ccg gcc atg gtg cag gag ctg cac cag ggc ggc cgg cgc       1530
Arg Asp Phe Pro Ala Met Val Gln Glu Leu His Gln Gly Gly Arg Arg           
            425                 430                 435                   

tac atg atg atc gtg gat cct gcc atc agc agc tcg ggc cct gcc ggg       1578
Tyr Met Met Ile Val Asp Pro Ala Ile Ser Ser Ser Gly Pro Ala Gly           
        440                 445                 450                       

agc tac agg ccc tac gac gag ggt ctg cgg agg ggg gtt ttc atc acc       1626
Ser Tyr Arg Pro Tyr Asp Glu Gly Leu Arg Arg Gly Val Phe Ile Thr           
    455                 460                 465                           

aac gag acc ggc cag ccg ctg att ggg aag gta tgg ccc ggg tcc act       1674
Asn Glu Thr Gly Gln Pro Leu Ile Gly Lys Val Trp Pro Gly Ser Thr           
470                 475                 480                 485           

gcc ttc ccc gac ttc acc aac ccc aca gcc ctg gcc tgg tgg gag gac       1722
Ala Phe Pro Asp Phe Thr Asn Pro Thr Ala Leu Ala Trp Trp Glu Asp           
                490                 495                 500               

atg gtg gct gag ttc cat gac cag gtg ccc ttc gac ggc atg tgg att       1770
Met Val Ala Glu Phe His Asp Gln Val Pro Phe Asp Gly Met Trp Ile           
            505                 510                 515                   

gac atg aac gag cct tcc aac ttc atc aga ggc tct gag gac ggc tgc       1818
Asp Met Asn Glu Pro Ser Asn Phe Ile Arg Gly Ser Glu Asp Gly Cys           
        520                 525                 530                       

ccc aac aat gag ctg gag aac cca ccc tac gtg cct ggg gtg gtt ggg       1866
Pro Asn Asn Glu Leu Glu Asn Pro Pro Tyr Val Pro Gly Val Val Gly           
    535                 540                 545                           

ggg acc ctc cag gcg gcc acc atc tgt gcc tcc agc cac cag ttt ctc       1914
Gly Thr Leu Gln Ala Ala Thr Ile Cys Ala Ser Ser His Gln Phe Leu           
550                 555                 560                 565           

tcc aca cac tac aac ctg cac aac ctc tac ggc ctg acc gaa gcc atc       1962
Ser Thr His Tyr Asn Leu His Asn Leu Tyr Gly Leu Thr Glu Ala Ile           
                570                 575                 580               

gcc tcc cac agg gcg ctg gtg aag gct cgg ggg aca cgc cca ttt gtg       2010
Ala Ser His Arg Ala Leu Val Lys Ala Arg Gly Thr Arg Pro Phe Val           
            585                 590                 595                   

atc tcc cgc tcg acc ttt gct ggc cac ggc cga tac gcc ggc cac tgg       2058
Ile Ser Arg Ser Thr Phe Ala Gly His Gly Arg Tyr Ala Gly His Trp           
        600                 605                 610                       

acg ggg gac gtg tgg agc tcc tgg gag cag ctc gcc tcc tcc gtg cca       2106
Thr Gly Asp Val Trp Ser Ser Trp Glu Gln Leu Ala Ser Ser Val Pro           
    615                 620                 625                           

gaa atc ctg cag ttt aac ctg ctg ggg gtg cct ctg gtc ggg gcc gac       2154
Glu Ile Leu Gln Phe Asn Leu Leu Gly Val Pro Leu Val Gly Ala Asp           
630                 635                 640                 645           

gtc tgc ggc ttc ctg ggc aac acc tca gag gag ctg tgt gtg cgc tgg       2202
Val Cys Gly Phe Leu Gly Asn Thr Ser Glu Glu Leu Cys Val Arg Trp           
                650                 655                 660               

acc cag ctg ggg gcc ttc tac ccc ttc atg cgg aac cac aac agc ctg       2250
Thr Gln Leu Gly Ala Phe Tyr Pro Phe Met Arg Asn His Asn Ser Leu           
            665                 670                 675                   

ctc agt ctg ccc cag gag ccg tac agc ttc agc gag ccg gcc cag cag       2298
Leu Ser Leu Pro Gln Glu Pro Tyr Ser Phe Ser Glu Pro Ala Gln Gln           
        680                 685                 690                       

gcc atg agg aag gcc ctc acc ctg cgc tac gca ctc ctc ccc cac ctc       2346
Ala Met Arg Lys Ala Leu Thr Leu Arg Tyr Ala Leu Leu Pro His Leu           
    695                 700                 705                           

tac aca ctg ttc cac cag gcc cac gtc gcg ggg gag acc gtg gcc cgg       2394
Tyr Thr Leu Phe His Gln Ala His Val Ala Gly Glu Thr Val Ala Arg           
710                 715                 720                 725           

ccc ctc ttc ctg gag ttc ccc aag gac tct agc acc tgg act gtg gac       2442
Pro Leu Phe Leu Glu Phe Pro Lys Asp Ser Ser Thr Trp Thr Val Asp           
                730                 735                 740               

cac cag ctc ctg tgg ggg gag gcc ctg ctc atc acc cca gtg ctc cag       2490
His Gln Leu Leu Trp Gly Glu Ala Leu Leu Ile Thr Pro Val Leu Gln           
            745                 750                 755                   

gcc ggg aag gcc gaa gtg act ggc tac ttc ccc ttg ggc aca tgg tac       2538
Ala Gly Lys Ala Glu Val Thr Gly Tyr Phe Pro Leu Gly Thr Trp Tyr           
        760                 765                 770                       

gac ctg cag acg gtg cca ata gag gcc ctt ggc agc ctc cca ccc cca       2586
Asp Leu Gln Thr Val Pro Ile Glu Ala Leu Gly Ser Leu Pro Pro Pro           
    775                 780                 785                           

cct gca gct ccc cgt gag cca gcc atc cac agc gag ggg cag tgg gtg       2634
Pro Ala Ala Pro Arg Glu Pro Ala Ile His Ser Glu Gly Gln Trp Val           
790                 795                 800                 805           

acg ctg ccg gcc ccc ctg gac acc atc aac gtc cac ctc cgg gct ggg       2682
Thr Leu Pro Ala Pro Leu Asp Thr Ile Asn Val His Leu Arg Ala Gly           
                810                 815                 820               

tac atc atc ccc ctg cag ggc cct ggc ctc aca acc aca gag tcc cgc       2730
Tyr Ile Ile Pro Leu Gln Gly Pro Gly Leu Thr Thr Thr Glu Ser Arg           
            825                 830                 835                   

cag cag ccc atg gcc ctg gct gtg gcc ctg acc aag ggt gga gag gcc       2778
Gln Gln Pro Met Ala Leu Ala Val Ala Leu Thr Lys Gly Gly Glu Ala           
        840                 845                 850                       

cga ggg gag ctg ttc tgg gac gat gga gag agc ctg gaa gtg ctg gag       2826
Arg Gly Glu Leu Phe Trp Asp Asp Gly Glu Ser Leu Glu Val Leu Glu           
    855                 860                 865                           

cga ggg gcc tac aca cag gtc atc ttc ctg gcc agg aat aac acg atc       2874
Arg Gly Ala Tyr Thr Gln Val Ile Phe Leu Ala Arg Asn Asn Thr Ile           
870                 875                 880                 885           

gtg aat gag ctg gta cgt gtg acc agt gag gga gct ggc ctg cag ctg       2922
Val Asn Glu Leu Val Arg Val Thr Ser Glu Gly Ala Gly Leu Gln Leu           
                890                 895                 900               

cag aag gtg act gtc ctg ggc gtg gcc acg gcg ccc cag cag gtc ctc       2970
Gln Lys Val Thr Val Leu Gly Val Ala Thr Ala Pro Gln Gln Val Leu           
            905                 910                 915                   

tcc aac ggt gtc cct gtc tcc aac ttc acc tac agc ccc gac acc aag       3018
Ser Asn Gly Val Pro Val Ser Asn Phe Thr Tyr Ser Pro Asp Thr Lys           
        920                 925                 930                       

gtc ctg gac atc tgt gtc tcg ctg ttg atg gga gag cag ttt ctc gtc       3066
Val Leu Asp Ile Cys Val Ser Leu Leu Met Gly Glu Gln Phe Leu Val           
    935                 940                 945                           

agc tgg tgt tag ccgggcggag tgtgttagtc tctccagagg gaggctggtt           3118
Ser Trp Cys                                                               
950                                                                       

ccccagggaa gcagagcctg tgtgcgggca gcagctgtgt gcgggcctgg gggttgcatg     3178

tgtcacctgg agctgggcac taaccattcc aagccgccgc atcgcttgtt tccacctcct     3238

gggccggggc tctggccccc aacgtgtcta ggagagcttt ctccctagat cgcactgtgg     3298

gccggggcct ggagggctgc tctgtgttaa taagattgta aggtttgccc tcctcacctg     3358

ttgccggcat gcgggtagta ttagccaccc ccctccatct gttcccagca ccggagaagg     3418

gggtgctcag gtggaggtgt ggggtatgca cctgagctcc tgcttcgcgc ctgctgctct     3478

gccccaacgc gaccgcttcc cggctgccca gagggctgga tgcctgccgg tccccgagca     3538

agcctgggaa ctcaggaaaa ttcacaggac ttgggagatt ctaaatctta agtgcaatta     3598

ttttaataaa aggggcattt ggaatc                                          3624


<210>  3
<211>  952
<212>  PRT
<213>  Homo sapiens

<400>  3

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


Ala Leu Val Ser Leu Ala Thr Ala Ala Leu Leu Gly His Ile Leu Leu 
            20                  25                  30          


His Asp Phe Leu Leu Val Pro Arg Glu Leu Ser Gly Ser Ser Pro Val 
        35                  40                  45              


Leu Glu Glu Thr His Pro Ala His Gln Gln Gly Ala Ser Arg Pro Gly 
    50                  55                  60                  


Pro Arg Asp Ala Gln Ala His Pro Gly Arg Pro Arg Ala Val Pro Thr 
65                  70                  75                  80  


Gln Cys Asp Val Pro Pro Asn Ser Arg Phe Asp Cys Ala Pro Asp Lys 
                85                  90                  95      


Ala Ile Thr Gln Glu Gln Cys Glu Ala Arg Gly Cys Cys Tyr Ile Pro 
            100                 105                 110         


Ala Lys Gln Gly Leu Gln Gly Ala Gln Met Gly Gln Pro Trp Cys Phe 
        115                 120                 125             


Phe Pro Pro Ser Tyr Pro Ser Tyr Lys Leu Glu Asn Leu Ser Ser Ser 
    130                 135                 140                 


Glu Met Gly Tyr Thr Ala Thr Leu Thr Arg Thr Thr Pro Thr Phe Phe 
145                 150                 155                 160 


Pro Lys Asp Ile Leu Thr Leu Arg Leu Asp Val Met Met Glu Thr Glu 
                165                 170                 175     


Asn Arg Leu His Phe Thr Ile Lys Asp Pro Ala Asn Arg Arg Tyr Glu 
            180                 185                 190         


Val Pro Leu Glu Thr Pro Arg Val His Ser Arg Ala Pro Ser Pro Leu 
        195                 200                 205             


Tyr Ser Val Glu Phe Ser Glu Glu Pro Phe Gly Val Ile Val His Arg 
    210                 215                 220                 


Gln Leu Asp Gly Arg Val Leu Leu Asn Thr Thr Val Ala Pro Leu Phe 
225                 230                 235                 240 


Phe Ala Asp Gln Phe Leu Gln Leu Ser Thr Ser Leu Pro Ser Gln Tyr 
                245                 250                 255     


Ile Thr Gly Leu Ala Glu His Leu Ser Pro Leu Met Leu Ser Thr Ser 
            260                 265                 270         


Trp Thr Arg Ile Thr Leu Trp Asn Arg Asp Leu Ala Pro Thr Pro Gly 
        275                 280                 285             


Ala Asn Leu Tyr Gly Ser His Pro Phe Tyr Leu Ala Leu Glu Asp Gly 
    290                 295                 300                 


Gly Ser Ala His Gly Val Phe Leu Leu Asn Ser Asn Ala Met Asp Val 
305                 310                 315                 320 


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


Leu Asp Val Tyr Ile Phe Leu Gly Pro Glu Pro Lys Ser Val Val Gln 
            340                 345                 350         


Gln Tyr Leu Asp Val Val Gly Tyr Pro Phe Met Pro Pro Tyr Trp Gly 
        355                 360                 365             


Leu Gly Phe His Leu Cys Arg Trp Gly Tyr Ser Ser Thr Ala Ile Thr 
    370                 375                 380                 


Arg Gln Val Val Glu Asn Met Thr Arg Ala His Phe Pro Leu Asp Val 
385                 390                 395                 400 


Gln Trp Asn Asp Leu Asp Tyr Met Asp Ser Arg Arg Asp Phe Thr Phe 
                405                 410                 415     


Asn Lys Asp Gly Phe Arg Asp Phe Pro Ala Met Val Gln Glu Leu His 
            420                 425                 430         


Gln Gly Gly Arg Arg Tyr Met Met Ile Val Asp Pro Ala Ile Ser Ser 
        435                 440                 445             


Ser Gly Pro Ala Gly Ser Tyr Arg Pro Tyr Asp Glu Gly Leu Arg Arg 
    450                 455                 460                 


Gly Val Phe Ile Thr Asn Glu Thr Gly Gln Pro Leu Ile Gly Lys Val 
465                 470                 475                 480 


Trp Pro Gly Ser Thr Ala Phe Pro Asp Phe Thr Asn Pro Thr Ala Leu 
                485                 490                 495     


Ala Trp Trp Glu Asp Met Val Ala Glu Phe His Asp Gln Val Pro Phe 
            500                 505                 510         


Asp Gly Met Trp Ile Asp Met Asn Glu Pro Ser Asn Phe Ile Arg Gly 
        515                 520                 525             


Ser Glu Asp Gly Cys Pro Asn Asn Glu Leu Glu Asn Pro Pro Tyr Val 
    530                 535                 540                 


Pro Gly Val Val Gly Gly Thr Leu Gln Ala Ala Thr Ile Cys Ala Ser 
545                 550                 555                 560 


Ser His Gln Phe Leu Ser Thr His Tyr Asn Leu His Asn Leu Tyr Gly 
                565                 570                 575     


Leu Thr Glu Ala Ile Ala Ser His Arg Ala Leu Val Lys Ala Arg Gly 
            580                 585                 590         


Thr Arg Pro Phe Val Ile Ser Arg Ser Thr Phe Ala Gly His Gly Arg 
        595                 600                 605             


Tyr Ala Gly His Trp Thr Gly Asp Val Trp Ser Ser Trp Glu Gln Leu 
    610                 615                 620                 


Ala Ser Ser Val Pro Glu Ile Leu Gln Phe Asn Leu Leu Gly Val Pro 
625                 630                 635                 640 


Leu Val Gly Ala Asp Val Cys Gly Phe Leu Gly Asn Thr Ser Glu Glu 
                645                 650                 655     


Leu Cys Val Arg Trp Thr Gln Leu Gly Ala Phe Tyr Pro Phe Met Arg 
            660                 665                 670         


Asn His Asn Ser Leu Leu Ser Leu Pro Gln Glu Pro Tyr Ser Phe Ser 
        675                 680                 685             


Glu Pro Ala Gln Gln Ala Met Arg Lys Ala Leu Thr Leu Arg Tyr Ala 
    690                 695                 700                 


Leu Leu Pro His Leu Tyr Thr Leu Phe His Gln Ala His Val Ala Gly 
705                 710                 715                 720 


Glu Thr Val Ala Arg Pro Leu Phe Leu Glu Phe Pro Lys Asp Ser Ser 
                725                 730                 735     


Thr Trp Thr Val Asp His Gln Leu Leu Trp Gly Glu Ala Leu Leu Ile 
            740                 745                 750         


Thr Pro Val Leu Gln Ala Gly Lys Ala Glu Val Thr Gly Tyr Phe Pro 
        755                 760                 765             


Leu Gly Thr Trp Tyr Asp Leu Gln Thr Val Pro Ile Glu Ala Leu Gly 
    770                 775                 780                 


Ser Leu Pro Pro Pro Pro Ala Ala Pro Arg Glu Pro Ala Ile His Ser 
785                 790                 795                 800 


Glu Gly Gln Trp Val Thr Leu Pro Ala Pro Leu Asp Thr Ile Asn Val 
                805                 810                 815     


His Leu Arg Ala Gly Tyr Ile Ile Pro Leu Gln Gly Pro Gly Leu Thr 
            820                 825                 830         


Thr Thr Glu Ser Arg Gln Gln Pro Met Ala Leu Ala Val Ala Leu Thr 
        835                 840                 845             


Lys Gly Gly Glu Ala Arg Gly Glu Leu Phe Trp Asp Asp Gly Glu Ser 
    850                 855                 860                 


Leu Glu Val Leu Glu Arg Gly Ala Tyr Thr Gln Val Ile Phe Leu Ala 
865                 870                 875                 880 


Arg Asn Asn Thr Ile Val Asn Glu Leu Val Arg Val Thr Ser Glu Gly 
                885                 890                 895     


Ala Gly Leu Gln Leu Gln Lys Val Thr Val Leu Gly Val Ala Thr Ala 
            900                 905                 910         


Pro Gln Gln Val Leu Ser Asn Gly Val Pro Val Ser Asn Phe Thr Tyr 
        915                 920                 925             


Ser Pro Asp Thr Lys Val Leu Asp Ile Cys Val Ser Leu Leu Met Gly 
    930                 935                 940                 


Glu Gln Phe Leu Val Ser Trp Cys 
945                 950         


<210>  4
<211>  952
<212>  PRT
<213>  Homo sapiens


<220>
<221>  MISC_FEATURE
<222>  (1)..(952)
<223>  Lysosomal alpha-glucosidase preproprotein [Homo sapiens];       NCBI Reference Sequence: NP_000143.2

<400>  4

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


Ala Leu Val Ser Leu Ala Thr Ala Ala Leu Leu Gly His Ile Leu Leu 
            20                  25                  30          


His Asp Phe Leu Leu Val Pro Arg Glu Leu Ser Gly Ser Ser Pro Val 
        35                  40                  45              


Leu Glu Glu Thr His Pro Ala His Gln Gln Gly Ala Ser Arg Pro Gly 
    50                  55                  60                  


Pro Arg Asp Ala Gln Ala His Pro Gly Arg Pro Arg Ala Val Pro Thr 
65                  70                  75                  80  


Gln Cys Asp Val Pro Pro Asn Ser Arg Phe Asp Cys Ala Pro Asp Lys 
                85                  90                  95      


Ala Ile Thr Gln Glu Gln Cys Glu Ala Arg Gly Cys Cys Tyr Ile Pro 
            100                 105                 110         


Ala Lys Gln Gly Leu Gln Gly Ala Gln Met Gly Gln Pro Trp Cys Phe 
        115                 120                 125             


Phe Pro Pro Ser Tyr Pro Ser Tyr Lys Leu Glu Asn Leu Ser Ser Ser 
    130                 135                 140                 


Glu Met Gly Tyr Thr Ala Thr Leu Thr Arg Thr Thr Pro Thr Phe Phe 
145                 150                 155                 160 


Pro Lys Asp Ile Leu Thr Leu Arg Leu Asp Val Met Met Glu Thr Glu 
                165                 170                 175     


Asn Arg Leu His Phe Thr Ile Lys Asp Pro Ala Asn Arg Arg Tyr Glu 
            180                 185                 190         


Val Pro Leu Glu Thr Pro His Val His Ser Arg Ala Pro Ser Pro Leu 
        195                 200                 205             


Tyr Ser Val Glu Phe Ser Glu Glu Pro Phe Gly Val Ile Val Arg Arg 
    210                 215                 220                 


Gln Leu Asp Gly Arg Val Leu Leu Asn Thr Thr Val Ala Pro Leu Phe 
225                 230                 235                 240 


Phe Ala Asp Gln Phe Leu Gln Leu Ser Thr Ser Leu Pro Ser Gln Tyr 
                245                 250                 255     


Ile Thr Gly Leu Ala Glu His Leu Ser Pro Leu Met Leu Ser Thr Ser 
            260                 265                 270         


Trp Thr Arg Ile Thr Leu Trp Asn Arg Asp Leu Ala Pro Thr Pro Gly 
        275                 280                 285             


Ala Asn Leu Tyr Gly Ser His Pro Phe Tyr Leu Ala Leu Glu Asp Gly 
    290                 295                 300                 


Gly Ser Ala His Gly Val Phe Leu Leu Asn Ser Asn Ala Met Asp Val 
305                 310                 315                 320 


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


Leu Asp Val Tyr Ile Phe Leu Gly Pro Glu Pro Lys Ser Val Val Gln 
            340                 345                 350         


Gln Tyr Leu Asp Val Val Gly Tyr Pro Phe Met Pro Pro Tyr Trp Gly 
        355                 360                 365             


Leu Gly Phe His Leu Cys Arg Trp Gly Tyr Ser Ser Thr Ala Ile Thr 
    370                 375                 380                 


Arg Gln Val Val Glu Asn Met Thr Arg Ala His Phe Pro Leu Asp Val 
385                 390                 395                 400 


Gln Trp Asn Asp Leu Asp Tyr Met Asp Ser Arg Arg Asp Phe Thr Phe 
                405                 410                 415     


Asn Lys Asp Gly Phe Arg Asp Phe Pro Ala Met Val Gln Glu Leu His 
            420                 425                 430         


Gln Gly Gly Arg Arg Tyr Met Met Ile Val Asp Pro Ala Ile Ser Ser 
        435                 440                 445             


Ser Gly Pro Ala Gly Ser Tyr Arg Pro Tyr Asp Glu Gly Leu Arg Arg 
    450                 455                 460                 


Gly Val Phe Ile Thr Asn Glu Thr Gly Gln Pro Leu Ile Gly Lys Val 
465                 470                 475                 480 


Trp Pro Gly Ser Thr Ala Phe Pro Asp Phe Thr Asn Pro Thr Ala Leu 
                485                 490                 495     


Ala Trp Trp Glu Asp Met Val Ala Glu Phe His Asp Gln Val Pro Phe 
            500                 505                 510         


Asp Gly Met Trp Ile Asp Met Asn Glu Pro Ser Asn Phe Ile Arg Gly 
        515                 520                 525             


Ser Glu Asp Gly Cys Pro Asn Asn Glu Leu Glu Asn Pro Pro Tyr Val 
    530                 535                 540                 


Pro Gly Val Val Gly Gly Thr Leu Gln Ala Ala Thr Ile Cys Ala Ser 
545                 550                 555                 560 


Ser His Gln Phe Leu Ser Thr His Tyr Asn Leu His Asn Leu Tyr Gly 
                565                 570                 575     


Leu Thr Glu Ala Ile Ala Ser His Arg Ala Leu Val Lys Ala Arg Gly 
            580                 585                 590         


Thr Arg Pro Phe Val Ile Ser Arg Ser Thr Phe Ala Gly His Gly Arg 
        595                 600                 605             


Tyr Ala Gly His Trp Thr Gly Asp Val Trp Ser Ser Trp Glu Gln Leu 
    610                 615                 620                 


Ala Ser Ser Val Pro Glu Ile Leu Gln Phe Asn Leu Leu Gly Val Pro 
625                 630                 635                 640 


Leu Val Gly Ala Asp Val Cys Gly Phe Leu Gly Asn Thr Ser Glu Glu 
                645                 650                 655     


Leu Cys Val Arg Trp Thr Gln Leu Gly Ala Phe Tyr Pro Phe Met Arg 
            660                 665                 670         


Asn His Asn Ser Leu Leu Ser Leu Pro Gln Glu Pro Tyr Ser Phe Ser 
        675                 680                 685             


Glu Pro Ala Gln Gln Ala Met Arg Lys Ala Leu Thr Leu Arg Tyr Ala 
    690                 695                 700                 


Leu Leu Pro His Leu Tyr Thr Leu Phe His Gln Ala His Val Ala Gly 
705                 710                 715                 720 


Glu Thr Val Ala Arg Pro Leu Phe Leu Glu Phe Pro Lys Asp Ser Ser 
                725                 730                 735     


Thr Trp Thr Val Asp His Gln Leu Leu Trp Gly Glu Ala Leu Leu Ile 
            740                 745                 750         


Thr Pro Val Leu Gln Ala Gly Lys Ala Glu Val Thr Gly Tyr Phe Pro 
        755                 760                 765             


Leu Gly Thr Trp Tyr Asp Leu Gln Thr Val Pro Val Glu Ala Leu Gly 
    770                 775                 780                 


Ser Leu Pro Pro Pro Pro Ala Ala Pro Arg Glu Pro Ala Ile His Ser 
785                 790                 795                 800 


Glu Gly Gln Trp Val Thr Leu Pro Ala Pro Leu Asp Thr Ile Asn Val 
                805                 810                 815     


His Leu Arg Ala Gly Tyr Ile Ile Pro Leu Gln Gly Pro Gly Leu Thr 
            820                 825                 830         


Thr Thr Glu Ser Arg Gln Gln Pro Met Ala Leu Ala Val Ala Leu Thr 
        835                 840                 845             


Lys Gly Gly Glu Ala Arg Gly Glu Leu Phe Trp Asp Asp Gly Glu Ser 
    850                 855                 860                 


Leu Glu Val Leu Glu Arg Gly Ala Tyr Thr Gln Val Ile Phe Leu Ala 
865                 870                 875                 880 


Arg Asn Asn Thr Ile Val Asn Glu Leu Val Arg Val Thr Ser Glu Gly 
                885                 890                 895     


Ala Gly Leu Gln Leu Gln Lys Val Thr Val Leu Gly Val Ala Thr Ala 
            900                 905                 910         


Pro Gln Gln Val Leu Ser Asn Gly Val Pro Val Ser Asn Phe Thr Tyr 
        915                 920                 925             


Ser Pro Asp Thr Lys Val Leu Asp Ile Cys Val Ser Leu Leu Met Gly 
    930                 935                 940                 


Glu Gln Phe Leu Val Ser Trp Cys 
945                 950         


