                         SEQUENCE LISTING

<110>  Amicus Therapeutics, Inc.
 
<120>  RECOMBINANT HUMAN ACID ALPHA-GLUCOSIDASE AND USES THEREOF

<130>  AMCS-013/02WO 337351-2416

<150>  US 63/162,683
<151>  2021-03-18

<150>  US 63/148,596
<151>  2021-02-11

<160>  7     

<170>  PatentIn version 3.5

<210>  1
<211>  952
<212>  PRT
<213>  Artificial Sequence

<220>
<223>  recombinant human acid alpha-glucosidase

<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>  Artificial Sequence

<220>
<223>  recombinant human acid alpha-glucosidase

<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 tctccaacca tgggagtgag gcacccgccc      240

tgctcccacc ggctcctggc cgtctgcgcc ctcgtgtcct tggcaaccgc tgcactcctg      300

gggcacatcc tactccatga tttcctgctg gttccccgag agctgagtgg ctcctcccca      360

gtcctggagg agactcaccc agctcaccag cagggagcca gcagaccagg gccccgggat      420

gcccaggcac accccggccg tcccagagca gtgcccacac agtgcgacgt cccccccaac      480

agccgcttcg attgcgcccc tgacaaggcc atcacccagg aacagtgcga ggcccgcggc      540

tgctgctaca tccctgcaaa gcaggggctg cagggagccc agatggggca gccctggtgc      600

ttcttcccac ccagctaccc cagctacaag ctggagaacc tgagctcctc tgaaatgggc      660

tacacggcca ccctgacccg taccaccccc accttcttcc ccaaggacat cctgaccctg      720

cggctggacg tgatgatgga gactgagaac cgcctccact tcacgatcaa agatccagct      780

aacaggcgct acgaggtgcc cttggagacc ccgcgtgtcc acagccgggc accgtcccca      840

ctctacagcg tggagttctc cgaggagccc ttcggggtga tcgtgcaccg gcagctggac      900

ggccgcgtgc tgctgaacac gacggtggcg cccctgttct ttgcggacca gttccttcag      960

ctgtccacct cgctgccctc gcagtatatc acaggcctcg ccgagcacct cagtcccctg     1020

atgctcagca ccagctggac caggatcacc ctgtggaacc gggaccttgc gcccacgccc     1080

ggtgcgaacc tctacgggtc tcaccctttc tacctggcgc tggaggacgg cgggtcggca     1140

cacggggtgt tcctgctaaa cagcaatgcc atggatgtgg tcctgcagcc gagccctgcc     1200

cttagctgga ggtcgacagg tgggatcctg gatgtctaca tcttcctggg cccagagccc     1260

aagagcgtgg tgcagcagta cctggacgtt gtgggatacc cgttcatgcc gccatactgg     1320

ggcctgggct tccacctgtg ccgctggggc tactcctcca ccgctatcac ccgccaggtg     1380

gtggagaaca tgaccagggc ccacttcccc ctggacgtcc aatggaacga cctggactac     1440

atggactccc ggagggactt cacgttcaac aaggatggct tccgggactt cccggccatg     1500

gtgcaggagc tgcaccaggg cggccggcgc tacatgatga tcgtggatcc tgccatcagc     1560

agctcgggcc ctgccgggag ctacaggccc tacgacgagg gtctgcggag gggggttttc     1620

atcaccaacg agaccggcca gccgctgatt gggaaggtat ggcccgggtc cactgccttc     1680

cccgacttca ccaaccccac agccctggcc tggtgggagg acatggtggc tgagttccat     1740

gaccaggtgc ccttcgacgg catgtggatt gacatgaacg agccttccaa cttcatcaga     1800

ggctctgagg acggctgccc caacaatgag ctggagaacc caccctacgt gcctggggtg     1860

gttgggggga ccctccaggc ggccaccatc tgtgcctcca gccaccagtt tctctccaca     1920

cactacaacc tgcacaacct ctacggcctg accgaagcca tcgcctccca cagggcgctg     1980

gtgaaggctc gggggacacg cccatttgtg atctcccgct cgacctttgc tggccacggc     2040

cgatacgccg gccactggac gggggacgtg tggagctcct gggagcagct cgcctcctcc     2100

gtgccagaaa tcctgcagtt taacctgctg ggggtgcctc tggtcggggc cgacgtctgc     2160

ggcttcctgg gcaacacctc agaggagctg tgtgtgcgct ggacccagct gggggccttc     2220

taccccttca tgcggaacca caacagcctg ctcagtctgc cccaggagcc gtacagcttc     2280

agcgagccgg cccagcaggc catgaggaag gccctcaccc tgcgctacgc actcctcccc     2340

cacctctaca cactgttcca ccaggcccac gtcgcggggg agaccgtggc ccggcccctc     2400

ttcctggagt tccccaagga ctctagcacc tggactgtgg accaccagct cctgtggggg     2460

gaggccctgc tcatcacccc agtgctccag gccgggaagg ccgaagtgac tggctacttc     2520

cccttgggca catggtacga cctgcagacg gtgccaatag aggcccttgg cagcctccca     2580

cccccacctg cagctccccg tgagccagcc atccacagcg aggggcagtg ggtgacgctg     2640

ccggcccccc tggacaccat caacgtccac ctccgggctg ggtacatcat ccccctgcag     2700

ggccctggcc tcacaaccac agagtcccgc cagcagccca tggccctggc tgtggccctg     2760

accaagggtg gagaggcccg aggggagctg ttctgggacg atggagagag cctggaagtg     2820

ctggagcgag gggcctacac acaggtcatc ttcctggcca ggaataacac gatcgtgaat     2880

gagctggtac gtgtgaccag tgagggagct ggcctgcagc tgcagaaggt gactgtcctg     2940

ggcgtggcca cggcgcccca gcaggtcctc tccaacggtg tccctgtctc caacttcacc     3000

tacagccccg acaccaaggt cctggacatc tgtgtctcgc tgttgatggg agagcagttt     3060

ctcgtcagct ggtgttagcc gggcggagtg tgttagtctc tccagaggga ggctggttcc     3120

ccagggaagc agagcctgtg tgcgggcagc agctgtgtgc gggcctgggg gttgcatgtg     3180

tcacctggag ctgggcacta accattccaa gccgccgcat cgcttgtttc cacctcctgg     3240

gccggggctc tggcccccaa cgtgtctagg agagctttct ccctagatcg cactgtgggc     3300

cggggcctgg agggctgctc tgtgttaata agattgtaag gtttgccctc ctcacctgtt     3360

gccggcatgc gggtagtatt agccaccccc ctccatctgt tcccagcacc ggagaagggg     3420

gtgctcaggt ggaggtgtgg ggtatgcacc tgagctcctg cttcgcgcct gctgctctgc     3480

cccaacgcga ccgcttcccg gctgcccaga gggctggatg cctgccggtc cccgagcaag     3540

cctgggaact caggaaaatt cacaggactt gggagattct aaatcttaag tgcaattatt     3600

ttaataaaag gggcatttgg aatc                                            3624


<210>  3
<211>  952
<212>  PRT
<213>  Artificial Sequence

<220>
<223>  recombinant human acid alpha-glucosidase

<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>  Artificial Sequence

<220>
<223>  recombinant human acid alpha-glucosidase

<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         


<210>  5
<211>  896
<212>  PRT
<213>  Artificial Sequence

<220>
<223>  recombinant human acid alpha-glucosidase

<400>  5

Gln Gln Gly Ala Ser Arg Pro Gly Pro Arg Asp Ala Gln Ala His Pro 
1               5                   10                  15      


Gly Arg Pro Arg Ala Val Pro Thr Gln Cys Asp Val Pro Pro Asn Ser 
            20                  25                  30          


Arg Phe Asp Cys Ala Pro Asp Lys Ala Ile Thr Gln Glu Gln Cys Glu 
        35                  40                  45              


Ala Arg Gly Cys Cys Tyr Ile Pro Ala Lys Gln Gly Leu Gln Gly Ala 
    50                  55                  60                  


Gln Met Gly Gln Pro Trp Cys Phe Phe Pro Pro Ser Tyr Pro Ser Tyr 
65                  70                  75                  80  


Lys Leu Glu Asn Leu Ser Ser Ser Glu Met Gly Tyr Thr Ala Thr Leu 
                85                  90                  95      


Thr Arg Thr Thr Pro Thr Phe Phe Pro Lys Asp Ile Leu Thr Leu Arg 
            100                 105                 110         


Leu Asp Val Met Met Glu Thr Glu Asn Arg Leu His Phe Thr Ile Lys 
        115                 120                 125             


Asp Pro Ala Asn Arg Arg Tyr Glu Val Pro Leu Glu Thr Pro Arg Val 
    130                 135                 140                 


His Ser Arg Ala Pro Ser Pro Leu Tyr Ser Val Glu Phe Ser Glu Glu 
145                 150                 155                 160 


Pro Phe Gly Val Ile Val His Arg Gln Leu Asp Gly Arg Val Leu Leu 
                165                 170                 175     


Asn Thr Thr Val Ala Pro Leu Phe Phe Ala Asp Gln Phe Leu Gln Leu 
            180                 185                 190         


Ser Thr Ser Leu Pro Ser Gln Tyr Ile Thr Gly Leu Ala Glu His Leu 
        195                 200                 205             


Ser Pro Leu Met Leu Ser Thr Ser Trp Thr Arg Ile Thr Leu Trp Asn 
    210                 215                 220                 


Arg Asp Leu Ala Pro Thr Pro Gly Ala Asn Leu Tyr Gly Ser His Pro 
225                 230                 235                 240 


Phe Tyr Leu Ala Leu Glu Asp Gly Gly Ser Ala His Gly Val Phe Leu 
                245                 250                 255     


Leu Asn Ser Asn Ala Met Asp Val Val Leu Gln Pro Ser Pro Ala Leu 
            260                 265                 270         


Ser Trp Arg Ser Thr Gly Gly Ile Leu Asp Val Tyr Ile Phe Leu Gly 
        275                 280                 285             


Pro Glu Pro Lys Ser Val Val Gln Gln Tyr Leu Asp Val Val Gly Tyr 
    290                 295                 300                 


Pro Phe Met Pro Pro Tyr Trp Gly Leu Gly Phe His Leu Cys Arg Trp 
305                 310                 315                 320 


Gly Tyr Ser Ser Thr Ala Ile Thr Arg Gln Val Val Glu Asn Met Thr 
                325                 330                 335     


Arg Ala His Phe Pro Leu Asp Val Gln Trp Asn Asp Leu Asp Tyr Met 
            340                 345                 350         


Asp Ser Arg Arg Asp Phe Thr Phe Asn Lys Asp Gly Phe Arg Asp Phe 
        355                 360                 365             


Pro Ala Met Val Gln Glu Leu His Gln Gly Gly Arg Arg Tyr Met Met 
    370                 375                 380                 


Ile Val Asp Pro Ala Ile Ser Ser Ser Gly Pro Ala Gly Ser Tyr Arg 
385                 390                 395                 400 


Pro Tyr Asp Glu Gly Leu Arg Arg Gly Val Phe Ile Thr Asn Glu Thr 
                405                 410                 415     


Gly Gln Pro Leu Ile Gly Lys Val Trp Pro Gly Ser Thr Ala Phe Pro 
            420                 425                 430         


Asp Phe Thr Asn Pro Thr Ala Leu Ala Trp Trp Glu Asp Met Val Ala 
        435                 440                 445             


Glu Phe His Asp Gln Val Pro Phe Asp Gly Met Trp Ile Asp Met Asn 
    450                 455                 460                 


Glu Pro Ser Asn Phe Ile Arg Gly Ser Glu Asp Gly Cys Pro Asn Asn 
465                 470                 475                 480 


Glu Leu Glu Asn Pro Pro Tyr Val Pro Gly Val Val Gly Gly Thr Leu 
                485                 490                 495     


Gln Ala Ala Thr Ile Cys Ala Ser Ser His Gln Phe Leu Ser Thr His 
            500                 505                 510         


Tyr Asn Leu His Asn Leu Tyr Gly Leu Thr Glu Ala Ile Ala Ser His 
        515                 520                 525             


Arg Ala Leu Val Lys Ala Arg Gly Thr Arg Pro Phe Val Ile Ser Arg 
    530                 535                 540                 


Ser Thr Phe Ala Gly His Gly Arg Tyr Ala Gly His Trp Thr Gly Asp 
545                 550                 555                 560 


Val Trp Ser Ser Trp Glu Gln Leu Ala Ser Ser Val Pro Glu Ile Leu 
                565                 570                 575     


Gln Phe Asn Leu Leu Gly Val Pro Leu Val Gly Ala Asp Val Cys Gly 
            580                 585                 590         


Phe Leu Gly Asn Thr Ser Glu Glu Leu Cys Val Arg Trp Thr Gln Leu 
        595                 600                 605             


Gly Ala Phe Tyr Pro Phe Met Arg Asn His Asn Ser Leu Leu Ser Leu 
    610                 615                 620                 


Pro Gln Glu Pro Tyr Ser Phe Ser Glu Pro Ala Gln Gln Ala Met Arg 
625                 630                 635                 640 


Lys Ala Leu Thr Leu Arg Tyr Ala Leu Leu Pro His Leu Tyr Thr Leu 
                645                 650                 655     


Phe His Gln Ala His Val Ala Gly Glu Thr Val Ala Arg Pro Leu Phe 
            660                 665                 670         


Leu Glu Phe Pro Lys Asp Ser Ser Thr Trp Thr Val Asp His Gln Leu 
        675                 680                 685             


Leu Trp Gly Glu Ala Leu Leu Ile Thr Pro Val Leu Gln Ala Gly Lys 
    690                 695                 700                 


Ala Glu Val Thr Gly Tyr Phe Pro Leu Gly Thr Trp Tyr Asp Leu Gln 
705                 710                 715                 720 


Thr Val Pro Ile Glu Ala Leu Gly Ser Leu Pro Pro Pro Pro Ala Ala 
                725                 730                 735     


Pro Arg Glu Pro Ala Ile His Ser Glu Gly Gln Trp Val Thr Leu Pro 
            740                 745                 750         


Ala Pro Leu Asp Thr Ile Asn Val His Leu Arg Ala Gly Tyr Ile Ile 
        755                 760                 765             


Pro Leu Gln Gly Pro Gly Leu Thr Thr Thr Glu Ser Arg Gln Gln Pro 
    770                 775                 780                 


Met Ala Leu Ala Val Ala Leu Thr Lys Gly Gly Glu Ala Arg Gly Glu 
785                 790                 795                 800 


Leu Phe Trp Asp Asp Gly Glu Ser Leu Glu Val Leu Glu Arg Gly Ala 
                805                 810                 815     


Tyr Thr Gln Val Ile Phe Leu Ala Arg Asn Asn Thr Ile Val Asn Glu 
            820                 825                 830         


Leu Val Arg Val Thr Ser Glu Gly Ala Gly Leu Gln Leu Gln Lys Val 
        835                 840                 845             


Thr Val Leu Gly Val Ala Thr Ala Pro Gln Gln Val Leu Ser Asn Gly 
    850                 855                 860                 


Val Pro Val Ser Asn Phe Thr Tyr Ser Pro Asp Thr Lys Val Leu Asp 
865                 870                 875                 880 


Ile Cys Val Ser Leu Leu Met Gly Glu Gln Phe Leu Val Ser Trp Cys 
                885                 890                 895     


<210>  6
<211>  896
<212>  PRT
<213>  Artificial Sequence

<220>
<223>  recombinant human acid alpha-glucosidase

<400>  6

Gln Gln Gly Ala Ser Arg Pro Gly Pro Arg Asp Ala Gln Ala His Pro 
1               5                   10                  15      


Gly Arg Pro Arg Ala Val Pro Thr Gln Cys Asp Val Pro Pro Asn Ser 
            20                  25                  30          


Arg Phe Asp Cys Ala Pro Asp Lys Ala Ile Thr Gln Glu Gln Cys Glu 
        35                  40                  45              


Ala Arg Gly Cys Cys Tyr Ile Pro Ala Lys Gln Gly Leu Gln Gly Ala 
    50                  55                  60                  


Gln Met Gly Gln Pro Trp Cys Phe Phe Pro Pro Ser Tyr Pro Ser Tyr 
65                  70                  75                  80  


Lys Leu Glu Asn Leu Ser Ser Ser Glu Met Gly Tyr Thr Ala Thr Leu 
                85                  90                  95      


Thr Arg Thr Thr Pro Thr Phe Phe Pro Lys Asp Ile Leu Thr Leu Arg 
            100                 105                 110         


Leu Asp Val Met Met Glu Thr Glu Asn Arg Leu His Phe Thr Ile Lys 
        115                 120                 125             


Asp Pro Ala Asn Arg Arg Tyr Glu Val Pro Leu Glu Thr Pro His Val 
    130                 135                 140                 


His Ser Arg Ala Pro Ser Pro Leu Tyr Ser Val Glu Phe Ser Glu Glu 
145                 150                 155                 160 


Pro Phe Gly Val Ile Val Arg Arg Gln Leu Asp Gly Arg Val Leu Leu 
                165                 170                 175     


Asn Thr Thr Val Ala Pro Leu Phe Phe Ala Asp Gln Phe Leu Gln Leu 
            180                 185                 190         


Ser Thr Ser Leu Pro Ser Gln Tyr Ile Thr Gly Leu Ala Glu His Leu 
        195                 200                 205             


Ser Pro Leu Met Leu Ser Thr Ser Trp Thr Arg Ile Thr Leu Trp Asn 
    210                 215                 220                 


Arg Asp Leu Ala Pro Thr Pro Gly Ala Asn Leu Tyr Gly Ser His Pro 
225                 230                 235                 240 


Phe Tyr Leu Ala Leu Glu Asp Gly Gly Ser Ala His Gly Val Phe Leu 
                245                 250                 255     


Leu Asn Ser Asn Ala Met Asp Val Val Leu Gln Pro Ser Pro Ala Leu 
            260                 265                 270         


Ser Trp Arg Ser Thr Gly Gly Ile Leu Asp Val Tyr Ile Phe Leu Gly 
        275                 280                 285             


Pro Glu Pro Lys Ser Val Val Gln Gln Tyr Leu Asp Val Val Gly Tyr 
    290                 295                 300                 


Pro Phe Met Pro Pro Tyr Trp Gly Leu Gly Phe His Leu Cys Arg Trp 
305                 310                 315                 320 


Gly Tyr Ser Ser Thr Ala Ile Thr Arg Gln Val Val Glu Asn Met Thr 
                325                 330                 335     


Arg Ala His Phe Pro Leu Asp Val Gln Trp Asn Asp Leu Asp Tyr Met 
            340                 345                 350         


Asp Ser Arg Arg Asp Phe Thr Phe Asn Lys Asp Gly Phe Arg Asp Phe 
        355                 360                 365             


Pro Ala Met Val Gln Glu Leu His Gln Gly Gly Arg Arg Tyr Met Met 
    370                 375                 380                 


Ile Val Asp Pro Ala Ile Ser Ser Ser Gly Pro Ala Gly Ser Tyr Arg 
385                 390                 395                 400 


Pro Tyr Asp Glu Gly Leu Arg Arg Gly Val Phe Ile Thr Asn Glu Thr 
                405                 410                 415     


Gly Gln Pro Leu Ile Gly Lys Val Trp Pro Gly Ser Thr Ala Phe Pro 
            420                 425                 430         


Asp Phe Thr Asn Pro Thr Ala Leu Ala Trp Trp Glu Asp Met Val Ala 
        435                 440                 445             


Glu Phe His Asp Gln Val Pro Phe Asp Gly Met Trp Ile Asp Met Asn 
    450                 455                 460                 


Glu Pro Ser Asn Phe Ile Arg Gly Ser Glu Asp Gly Cys Pro Asn Asn 
465                 470                 475                 480 


Glu Leu Glu Asn Pro Pro Tyr Val Pro Gly Val Val Gly Gly Thr Leu 
                485                 490                 495     


Gln Ala Ala Thr Ile Cys Ala Ser Ser His Gln Phe Leu Ser Thr His 
            500                 505                 510         


Tyr Asn Leu His Asn Leu Tyr Gly Leu Thr Glu Ala Ile Ala Ser His 
        515                 520                 525             


Arg Ala Leu Val Lys Ala Arg Gly Thr Arg Pro Phe Val Ile Ser Arg 
    530                 535                 540                 


Ser Thr Phe Ala Gly His Gly Arg Tyr Ala Gly His Trp Thr Gly Asp 
545                 550                 555                 560 


Val Trp Ser Ser Trp Glu Gln Leu Ala Ser Ser Val Pro Glu Ile Leu 
                565                 570                 575     


Gln Phe Asn Leu Leu Gly Val Pro Leu Val Gly Ala Asp Val Cys Gly 
            580                 585                 590         


Phe Leu Gly Asn Thr Ser Glu Glu Leu Cys Val Arg Trp Thr Gln Leu 
        595                 600                 605             


Gly Ala Phe Tyr Pro Phe Met Arg Asn His Asn Ser Leu Leu Ser Leu 
    610                 615                 620                 


Pro Gln Glu Pro Tyr Ser Phe Ser Glu Pro Ala Gln Gln Ala Met Arg 
625                 630                 635                 640 


Lys Ala Leu Thr Leu Arg Tyr Ala Leu Leu Pro His Leu Tyr Thr Leu 
                645                 650                 655     


Phe His Gln Ala His Val Ala Gly Glu Thr Val Ala Arg Pro Leu Phe 
            660                 665                 670         


Leu Glu Phe Pro Lys Asp Ser Ser Thr Trp Thr Val Asp His Gln Leu 
        675                 680                 685             


Leu Trp Gly Glu Ala Leu Leu Ile Thr Pro Val Leu Gln Ala Gly Lys 
    690                 695                 700                 


Ala Glu Val Thr Gly Tyr Phe Pro Leu Gly Thr Trp Tyr Asp Leu Gln 
705                 710                 715                 720 


Thr Val Pro Val Glu Ala Leu Gly Ser Leu Pro Pro Pro Pro Ala Ala 
                725                 730                 735     


Pro Arg Glu Pro Ala Ile His Ser Glu Gly Gln Trp Val Thr Leu Pro 
            740                 745                 750         


Ala Pro Leu Asp Thr Ile Asn Val His Leu Arg Ala Gly Tyr Ile Ile 
        755                 760                 765             


Pro Leu Gln Gly Pro Gly Leu Thr Thr Thr Glu Ser Arg Gln Gln Pro 
    770                 775                 780                 


Met Ala Leu Ala Val Ala Leu Thr Lys Gly Gly Glu Ala Arg Gly Glu 
785                 790                 795                 800 


Leu Phe Trp Asp Asp Gly Glu Ser Leu Glu Val Leu Glu Arg Gly Ala 
                805                 810                 815     


Tyr Thr Gln Val Ile Phe Leu Ala Arg Asn Asn Thr Ile Val Asn Glu 
            820                 825                 830         


Leu Val Arg Val Thr Ser Glu Gly Ala Gly Leu Gln Leu Gln Lys Val 
        835                 840                 845             


Thr Val Leu Gly Val Ala Thr Ala Pro Gln Gln Val Leu Ser Asn Gly 
    850                 855                 860                 


Val Pro Val Ser Asn Phe Thr Tyr Ser Pro Asp Thr Lys Val Leu Asp 
865                 870                 875                 880 


Ile Cys Val Ser Leu Leu Met Gly Glu Gln Phe Leu Val Ser Trp Cys 
                885                 890                 895     


<210>  7
<211>  6
<212>  PRT
<213>  Unknown

<220>
<223>  conserved hexapeptide

<400>  7

Trp Ile Asp Met Asn Glu 
1               5       


