                               SEQUENCE LISTING

<110> ALEXION PHARMACEUTICALS, INC.
 
<120> METHODS FOR REDUCING LIVER FIBROSIS AND TREATING LYSOSOMAL 
      ACID LIPASE DEFICIENCY IN PATIENTS BASED ON ISHAK FIBROSIS 
      STAGE

<130> AXJ-221PC

<140>
<141>

<150> 62/456,511
<151> 2017-02-08

<150> 62/420,233
<151> 2016-11-10

<150> 62/402,183
<151> 2016-09-30

<160> 2     

<170> PatentIn version 3.5

<210> 1
<211> 378
<212> PRT
<213> Homo sapiens

<400> 1
Ser Gly Gly Lys Leu Thr Ala Val Asp Pro Glu Thr Asn Met Asn Val 
1               5                   10                  15      


Ser Glu Ile Ile Ser Tyr Trp Gly Phe Pro Ser Glu Glu Tyr Leu Val 
            20                  25                  30          


Glu Thr Glu Asp Gly Tyr Ile Leu Cys Leu Asn Arg Ile Pro His Gly 
        35                  40                  45              


Arg Lys Asn His Ser Asp Lys Gly Pro Lys Pro Val Val Phe Leu Gln 
    50                  55                  60                  


His Gly Leu Leu Ala Asp Ser Ser Asn Trp Val Thr Asn Leu Ala Asn 
65                  70                  75                  80  


Ser Ser Leu Gly Phe Ile Leu Ala Asp Ala Gly Phe Asp Val Trp Met 
                85                  90                  95      


Gly Asn Ser Arg Gly Asn Thr Trp Ser Arg Lys His Lys Thr Leu Ser 
            100                 105                 110         


Val Ser Gln Asp Glu Phe Trp Ala Phe Ser Tyr Asp Glu Met Ala Lys 
        115                 120                 125             


Tyr Asp Leu Pro Ala Ser Ile Asn Phe Ile Leu Asn Lys Thr Gly Gln 
    130                 135                 140                 


Glu Gln Val Tyr Tyr Val Gly His Ser Gln Gly Thr Thr Ile Gly Phe 
145                 150                 155                 160 


Ile Ala Phe Ser Gln Ile Pro Glu Leu Ala Lys Arg Ile Lys Met Phe 
                165                 170                 175     


Phe Ala Leu Gly Pro Val Ala Ser Val Ala Phe Cys Thr Ser Pro Met 
            180                 185                 190         


Ala Lys Leu Gly Arg Leu Pro Asp His Leu Ile Lys Asp Leu Phe Gly 
        195                 200                 205             


Asp Lys Glu Phe Leu Pro Gln Ser Ala Phe Leu Lys Trp Leu Gly Thr 
    210                 215                 220                 


His Val Cys Thr His Val Ile Leu Lys Glu Leu Cys Gly Asn Leu Cys 
225                 230                 235                 240 


Phe Leu Leu Cys Gly Phe Asn Glu Arg Asn Leu Asn Met Ser Arg Val 
                245                 250                 255     


Asp Val Tyr Thr Thr His Ser Pro Ala Gly Thr Ser Val Gln Asn Met 
            260                 265                 270         


Leu His Trp Ser Gln Ala Val Lys Phe Gln Lys Phe Gln Ala Phe Asp 
        275                 280                 285             


Trp Gly Ser Ser Ala Lys Asn Tyr Phe His Tyr Asn Gln Ser Tyr Pro 
    290                 295                 300                 


Pro Thr Tyr Asn Val Lys Asp Met Leu Val Pro Thr Ala Val Trp Ser 
305                 310                 315                 320 


Gly Gly His Asp Trp Leu Ala Asp Val Tyr Asp Val Asn Ile Leu Leu 
                325                 330                 335     


Thr Gln Ile Thr Asn Leu Val Phe His Glu Ser Ile Pro Glu Trp Glu 
            340                 345                 350         


His Leu Asp Phe Ile Trp Gly Leu Asp Ala Pro Trp Arg Leu Tyr Asn 
        355                 360                 365             


Lys Ile Ile Asn Leu Met Arg Lys Tyr Gln 
    370                 375             


<210> 2
<211> 21
<212> PRT
<213> Homo sapiens

<400> 2
Met Lys Met Arg Phe Leu Gly Leu Val Val Cys Leu Val Leu Trp Thr 
1               5                   10                  15      


Leu His Ser Glu Gly 
            20      


