                         SEQUENCE LISTING

<110>  BIOMARIN PHARMACEUTICAL INC.
 
<120>  METHODS FOR TREATING CLN2 DISEASE IN PEDIATRIC SUBJECTS

<130>  30610/54735

<150>  US 62/893,535
<151>  2019-08-29

<160>  2     

<170>  PatentIn version 3.5

<210>  1
<211>  544
<212>  PRT
<213>  Homo sapiens

<400>  1

Ser Tyr Ser Pro Glu Pro Asp Gln Arg Arg Thr Leu Pro Pro Gly Trp 
1               5                   10                  15      


Val Ser Leu Gly Arg Ala Asp Pro Glu Glu Glu Leu Ser Leu Thr Phe 
            20                  25                  30          


Ala Leu Arg Gln Gln Asn Val Glu Arg Leu Ser Glu Leu Val Gln Ala 
        35                  40                  45              


Val Ser Asp Pro Ser Ser Pro Gln Tyr Gly Lys Tyr Leu Thr Leu Glu 
    50                  55                  60                  


Asn Val Ala Asp Leu Val Arg Pro Ser Pro Leu Thr Leu His Thr Val 
65                  70                  75                  80  


Gln Lys Trp Leu Leu Ala Ala Gly Ala Gln Lys Cys His Ser Val Ile 
                85                  90                  95      


Thr Gln Asp Phe Leu Thr Cys Trp Leu Ser Ile Arg Gln Ala Glu Leu 
            100                 105                 110         


Leu Leu Pro Gly Ala Glu Phe His His Tyr Val Gly Gly Pro Thr Glu 
        115                 120                 125             


Thr His Val Val Arg Ser Pro His Pro Tyr Gln Leu Pro Gln Ala Leu 
    130                 135                 140                 


Ala Pro His Val Asp Phe Val Gly Gly Leu His Arg Phe Pro Pro Thr 
145                 150                 155                 160 


Ser Ser Leu Arg Gln Arg Pro Glu Pro Gln Val Thr Gly Thr Val Gly 
                165                 170                 175     


Leu His Leu Gly Val Thr Pro Ser Val Ile Arg Lys Arg Tyr Asn Leu 
            180                 185                 190         


Thr Ser Gln Asp Val Gly Ser Gly Thr Ser Asn Asn Ser Gln Ala Cys 
        195                 200                 205             


Ala Gln Phe Leu Glu Gln Tyr Phe His Asp Ser Asp Leu Ala Gln Phe 
    210                 215                 220                 


Met Arg Leu Phe Gly Gly Asn Phe Ala His Gln Ala Ser Val Ala Arg 
225                 230                 235                 240 


Val Val Gly Gln Gln Gly Arg Gly Arg Ala Gly Ile Glu Ala Ser Leu 
                245                 250                 255     


Asp Val Gln Tyr Leu Met Ser Ala Gly Ala Asn Ile Ser Thr Trp Val 
            260                 265                 270         


Tyr Ser Ser Pro Gly Arg His Glu Gly Gln Glu Pro Phe Leu Gln Trp 
        275                 280                 285             


Leu Met Leu Leu Ser Asn Glu Ser Ala Leu Pro His Val His Thr Val 
    290                 295                 300                 


Ser Tyr Gly Asp Asp Glu Asp Ser Leu Ser Ser Ala Tyr Ile Gln Arg 
305                 310                 315                 320 


Val Asn Thr Glu Leu Met Lys Ala Ala Ala Arg Gly Leu Thr Leu Leu 
                325                 330                 335     


Phe Ala Ser Gly Asp Ser Gly Ala Gly Cys Trp Ser Val Ser Gly Arg 
            340                 345                 350         


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


Val Gly Gly Thr Ser Phe Gln Glu Pro Phe Leu Ile Thr Asn Glu Ile 
    370                 375                 380                 


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


Ser Tyr Gln Glu Glu Ala Val Thr Lys Phe Leu Ser Ser Ser Pro His 
                405                 410                 415     


Leu Pro Pro Ser Ser Tyr Phe Asn Ala Ser Gly Arg Ala Tyr Pro Asp 
            420                 425                 430         


Val Ala Ala Leu Ser Asp Gly Tyr Trp Val Val Ser Asn Arg Val Pro 
        435                 440                 445             


Ile Pro Trp Val Ser Gly Thr Ser Ala Ser Thr Pro Val Phe Gly Gly 
    450                 455                 460                 


Ile Leu Ser Leu Ile Asn Glu His Arg Ile Leu Ser Gly Arg Pro Pro 
465                 470                 475                 480 


Leu Gly Phe Leu Asn Pro Arg Leu Tyr Gln Gln His Gly Ala Gly Leu 
                485                 490                 495     


Phe Asp Val Thr Arg Gly Cys His Glu Ser Cys Leu Asp Glu Glu Val 
            500                 505                 510         


Glu Gly Gln Gly Phe Cys Ser Gly Pro Gly Trp Asp Pro Val Thr Gly 
        515                 520                 525             


Trp Gly Thr Pro Asn Phe Pro Ala Leu Leu Lys Thr Leu Leu Asn Pro 
    530                 535                 540                 


<210>  2
<211>  368
<212>  PRT
<213>  Homo sapiens

<400>  2

Leu His Leu Gly Val Thr Pro Ser Val Ile Arg Lys Arg Tyr Asn Leu 
1               5                   10                  15      


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


Ala Gln Phe Leu Glu Gln Tyr Phe His Asp Ser Asp Leu Ala Gln Phe 
        35                  40                  45              


Met Arg Leu Phe Gly Gly Asn Phe Ala His Gln Ala Ser Val Ala Arg 
    50                  55                  60                  


Val Val Gly Gln Gln Gly Arg Gly Arg Ala Gly Ile Glu Ala Ser Leu 
65                  70                  75                  80  


Asp Val Gln Tyr Leu Met Ser Ala Gly Ala Asn Ile Ser Thr Trp Val 
                85                  90                  95      


Tyr Ser Ser Pro Gly Arg His Glu Gly Gln Glu Pro Phe Leu Gln Trp 
            100                 105                 110         


Leu Met Leu Leu Ser Asn Glu Ser Ala Leu Pro His Val His Thr Val 
        115                 120                 125             


Ser Tyr Gly Asp Asp Glu Asp Ser Leu Ser Ser Ala Tyr Ile Gln Arg 
    130                 135                 140                 


Val Asn Thr Glu Leu Met Lys Ala Ala Ala Arg Gly Leu Thr Leu Leu 
145                 150                 155                 160 


Phe Ala Ser Gly Asp Ser Gly Ala Gly Cys Trp Ser Val Ser Gly Arg 
                165                 170                 175     


His Gln Phe Arg Pro Thr Phe Pro Ala Ser Ser Pro Tyr Val Thr Thr 
            180                 185                 190         


Val Gly Gly Thr Ser Phe Gln Glu Pro Phe Leu Ile Thr Asn Glu Ile 
        195                 200                 205             


Val Asp Tyr Ile Ser Gly Gly Gly Phe Ser Asn Val Phe Pro Arg Pro 
    210                 215                 220                 


Ser Tyr Gln Glu Glu Ala Val Thr Lys Phe Leu Ser Ser Ser Pro His 
225                 230                 235                 240 


Leu Pro Pro Ser Ser Tyr Phe Asn Ala Ser Gly Arg Ala Tyr Pro Asp 
                245                 250                 255     


Val Ala Ala Leu Ser Asp Gly Tyr Trp Val Val Ser Asn Arg Val Pro 
            260                 265                 270         


Ile Pro Trp Val Ser Gly Thr Ser Ala Ser Thr Pro Val Phe Gly Gly 
        275                 280                 285             


Ile Leu Ser Leu Ile Asn Glu His Arg Ile Leu Ser Gly Arg Pro Pro 
    290                 295                 300                 


Leu Gly Phe Leu Asn Pro Arg Leu Tyr Gln Gln His Gly Ala Gly Leu 
305                 310                 315                 320 


Phe Asp Val Thr Arg Gly Cys His Glu Ser Cys Leu Asp Glu Glu Val 
                325                 330                 335     


Glu Gly Gln Gly Phe Cys Ser Gly Pro Gly Trp Asp Pro Val Thr Gly 
            340                 345                 350         


Trp Gly Thr Pro Asn Phe Pro Ala Leu Leu Lys Thr Leu Leu Asn Pro 
        355                 360                 365             


