                         SEQUENCE LISTING

<110>  Shire Human Genetic Therapies, Inc.
 
<120>  RECOMBINANT HUMAN C1 ESTERASE INHIBITOR AND USES THEREOF

<130>  SHR-1234WO

<150>  62/257,711
<151>  2015-11-19

<160>  4     

<170>  PatentIn version 3.5

<210>  1
<211>  478
<212>  PRT
<213>  Homo sapiens

<400>  1

Asn Pro Asn Ala Thr Ser Ser Ser Ser Gln Asp Pro Glu Ser Leu Gln 
1               5                   10                  15      


Asp Arg Gly Glu Gly Lys Val Ala Thr Thr Val Ile Ser Lys Met Leu 
            20                  25                  30          


Phe Val Glu Pro Ile Leu Glu Val Ser Ser Leu Pro Thr Thr Asn Ser 
        35                  40                  45              


Thr Thr Asn Ser Ala Thr Lys Ile Thr Ala Asn Thr Thr Asp Glu Pro 
    50                  55                  60                  


Thr Thr Gln Pro Thr Thr Glu Pro Thr Thr Gln Pro Thr Ile Gln Pro 
65                  70                  75                  80  


Thr Gln Pro Thr Thr Gln Leu Pro Thr Asp Ser Pro Thr Gln Pro Thr 
                85                  90                  95      


Thr Gly Ser Phe Cys Pro Gly Pro Val Thr Leu Cys Ser Asp Leu Glu 
            100                 105                 110         


Ser His Ser Thr Glu Ala Val Leu Gly Asp Ala Leu Val Asp Phe Ser 
        115                 120                 125             


Leu Lys Leu Tyr His Ala Phe Ser Ala Met Lys Lys Val Glu Thr Asn 
    130                 135                 140                 


Met Ala Phe Ser Pro Phe Ser Ile Ala Ser Leu Leu Thr Gln Val Leu 
145                 150                 155                 160 


Leu Gly Ala Gly Glu Asn Thr Lys Thr Asn Leu Glu Ser Ile Leu Ser 
                165                 170                 175     


Tyr Pro Lys Asp Phe Thr Cys Val His Gln Ala Leu Lys Gly Phe Thr 
            180                 185                 190         


Thr Lys Gly Val Thr Ser Val Ser Gln Ile Phe His Ser Pro Asp Leu 
        195                 200                 205             


Ala Ile Arg Asp Thr Phe Val Asn Ala Ser Arg Thr Leu Tyr Ser Ser 
    210                 215                 220                 


Ser Pro Arg Val Leu Ser Asn Asn Ser Asp Ala Asn Leu Glu Leu Ile 
225                 230                 235                 240 


Asn Thr Trp Val Ala Lys Asn Thr Asn Asn Lys Ile Ser Arg Leu Leu 
                245                 250                 255     


Asp Ser Leu Pro Ser Asp Thr Arg Leu Val Leu Leu Asn Ala Ile Tyr 
            260                 265                 270         


Leu Ser Ala Lys Trp Lys Thr Thr Phe Asp Pro Lys Lys Thr Arg Met 
        275                 280                 285             


Glu Pro Phe His Phe Lys Asn Ser Val Ile Lys Val Pro Met Met Asn 
    290                 295                 300                 


Ser Lys Lys Tyr Pro Val Ala His Phe Ile Asp Gln Thr Leu Lys Ala 
305                 310                 315                 320 


Lys Val Gly Gln Leu Gln Leu Ser His Asn Leu Ser Leu Val Ile Leu 
                325                 330                 335     


Val Pro Gln Asn Leu Lys His Arg Leu Glu Asp Met Glu Gln Ala Leu 
            340                 345                 350         


Ser Pro Ser Val Phe Lys Ala Ile Met Glu Lys Leu Glu Met Ser Lys 
        355                 360                 365             


Phe Gln Pro Thr Leu Leu Thr Leu Pro Arg Ile Lys Val Thr Thr Ser 
    370                 375                 380                 


Gln Asp Met Leu Ser Ile Met Glu Lys Leu Glu Phe Phe Asp Phe Ser 
385                 390                 395                 400 


Tyr Asp Leu Asn Leu Cys Gly Leu Thr Glu Asp Pro Asp Leu Gln Val 
                405                 410                 415     


Ser Ala Met Gln His Gln Thr Val Leu Glu Leu Thr Glu Thr Gly Val 
            420                 425                 430         


Glu Ala Ala Ala Ala Ser Ala Ile Ser Val Ala Arg Thr Leu Leu Val 
        435                 440                 445             


Phe Glu Val Gln Gln Pro Phe Leu Phe Val Leu Trp Asp Gln Gln His 
    450                 455                 460                 


Lys Phe Pro Val Phe Met Gly Arg Val Tyr Asp Pro Arg Ala 
465                 470                 475             


<210>  2
<211>  478
<212>  PRT
<213>  Artificial Sequence

<220>
<223>  Synthetic Polypeptide

<400>  2

Asn Pro Asn Ala Thr Ser Ser Ser Ser Gln Asp Pro Glu Ser Leu Gln 
1               5                   10                  15      


Asp Arg Gly Glu Gly Lys Val Ala Thr Thr Val Ile Ser Lys Met Leu 
            20                  25                  30          


Phe Val Glu Pro Ile Leu Glu Val Ser Ser Leu Pro Thr Thr Asn Ser 
        35                  40                  45              


Thr Thr Asn Ser Ala Thr Lys Ile Thr Ala Asn Thr Thr Asp Glu Pro 
    50                  55                  60                  


Thr Thr Gln Pro Thr Thr Glu Pro Thr Thr Gln Pro Thr Ile Gln Pro 
65                  70                  75                  80  


Thr Gln Pro Thr Thr Gln Leu Pro Thr Asp Ser Pro Thr Gln Pro Thr 
                85                  90                  95      


Thr Gly Ser Phe Cys Pro Gly Pro Val Thr Leu Cys Ser Asp Leu Glu 
            100                 105                 110         


Ser His Ser Thr Glu Ala Val Leu Gly Asp Ala Leu Val Asp Phe Ser 
        115                 120                 125             


Leu Lys Leu Tyr His Ala Phe Ser Ala Met Lys Lys Val Glu Thr Asn 
    130                 135                 140                 


Met Ala Phe Ser Pro Phe Ser Ile Ala Ser Leu Leu Thr Gln Val Leu 
145                 150                 155                 160 


Leu Gly Ala Gly Glu Asn Thr Lys Thr Asn Leu Glu Ser Ile Leu Ser 
                165                 170                 175     


Tyr Pro Lys Asp Phe Thr Cys Val His Gln Ala Leu Lys Gly Phe Thr 
            180                 185                 190         


Thr Lys Gly Val Thr Ser Val Ser Gln Ile Phe His Ser Pro Asp Leu 
        195                 200                 205             


Ala Ile Arg Asp Thr Phe Val Asn Ala Ser Arg Thr Leu Tyr Ser Ser 
    210                 215                 220                 


Ser Pro Arg Val Leu Ser Asn Asn Ser Asp Ala Asn Leu Glu Leu Ile 
225                 230                 235                 240 


Asn Thr Trp Val Ala Lys Asn Thr Asn Asn Lys Ile Ser Arg Leu Leu 
                245                 250                 255     


Asp Ser Leu Pro Ser Asp Thr Arg Leu Val Leu Leu Asn Ala Ile Tyr 
            260                 265                 270         


Leu Ser Ala Lys Trp Lys Thr Thr Phe Asp Pro Lys Lys Thr Arg Met 
        275                 280                 285             


Glu Pro Phe His Phe Lys Asn Ser Val Ile Lys Val Pro Met Met Asn 
    290                 295                 300                 


Ser Lys Lys Tyr Pro Val Ala His Phe Ile Asp Gln Thr Leu Lys Ala 
305                 310                 315                 320 


Lys Val Gly Gln Leu Gln Leu Ser His Asn Leu Ser Leu Val Ile Leu 
                325                 330                 335     


Val Pro Gln Asn Leu Lys His Arg Leu Glu Asp Met Glu Gln Ala Leu 
            340                 345                 350         


Ser Pro Ser Val Phe Lys Ala Ile Met Glu Lys Leu Glu Met Ser Lys 
        355                 360                 365             


Phe Gln Pro Thr Leu Leu Thr Leu Pro Arg Ile Lys Val Thr Thr Ser 
    370                 375                 380                 


Gln Asp Met Leu Ser Ile Met Glu Lys Leu Glu Phe Phe Asp Phe Ser 
385                 390                 395                 400 


Tyr Asp Leu Asn Leu Cys Gly Leu Thr Glu Asp Pro Asp Leu Gln Val 
                405                 410                 415     


Ser Ala Met Gln His Gln Thr Val Leu Glu Leu Thr Glu Thr Gly Val 
            420                 425                 430         


Glu Ala Ala Ala Ala Ser Ala Ile Ser Val Ala Arg Thr Leu Leu Val 
        435                 440                 445             


Phe Glu Val Gln Gln Pro Phe Leu Phe Val Leu Trp Asp Gln Gln His 
    450                 455                 460                 


Lys Phe Pro Val Phe Met Gly Arg Val Tyr Asp Pro Arg Ala 
465                 470                 475             


<210>  3
<211>  381
<212>  PRT
<213>  Artificial Sequence

<220>
<223>  Synthetic Polypeptide

<400>  3

Gly Ser Phe Cys Pro Gly Pro Val Thr Leu Cys Ser Asp Leu Glu Ser 
1               5                   10                  15      


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


Lys Leu Tyr His Ala Phe Ser Ala Met Lys Lys Val Glu Thr Asn Met 
        35                  40                  45              


Ala Phe Ser Pro Phe Ser Ile Ala Ser Leu Leu Thr Gln Val Leu Leu 
    50                  55                  60                  


Gly Ala Gly Glu Asn Thr Lys Thr Asn Leu Glu Ser Ile Leu Ser Tyr 
65                  70                  75                  80  


Pro Lys Asp Phe Thr Cys Val His Gln Ala Leu Lys Gly Phe Thr Thr 
                85                  90                  95      


Lys Gly Val Thr Ser Val Ser Gln Ile Phe His Ser Pro Asp Leu Ala 
            100                 105                 110         


Ile Arg Asp Thr Phe Val Asn Ala Ser Arg Thr Leu Tyr Ser Ser Ser 
        115                 120                 125             


Pro Arg Val Leu Ser Asn Asn Ser Asp Ala Asn Leu Glu Leu Ile Asn 
    130                 135                 140                 


Thr Trp Val Ala Lys Asn Thr Asn Asn Lys Ile Ser Arg Leu Leu Asp 
145                 150                 155                 160 


Ser Leu Pro Ser Asp Thr Arg Leu Val Leu Leu Asn Ala Ile Tyr Leu 
                165                 170                 175     


Ser Ala Lys Trp Lys Thr Thr Phe Asp Pro Lys Lys Thr Arg Met Glu 
            180                 185                 190         


Pro Phe His Phe Lys Asn Ser Val Ile Lys Val Pro Met Met Asn Ser 
        195                 200                 205             


Lys Lys Tyr Pro Val Ala His Phe Ile Asp Gln Thr Leu Lys Ala Lys 
    210                 215                 220                 


Val Gly Gln Leu Gln Leu Ser His Asn Leu Ser Leu Val Ile Leu Val 
225                 230                 235                 240 


Pro Gln Asn Leu Lys His Arg Leu Glu Asp Met Glu Gln Ala Leu Ser 
                245                 250                 255     


Pro Ser Val Phe Lys Ala Ile Met Glu Lys Leu Glu Met Ser Lys Phe 
            260                 265                 270         


Gln Pro Thr Leu Leu Thr Leu Pro Arg Ile Lys Val Thr Thr Ser Gln 
        275                 280                 285             


Asp Met Leu Ser Ile Met Glu Lys Leu Glu Phe Phe Asp Phe Ser Tyr 
    290                 295                 300                 


Asp Leu Asn Leu Cys Gly Leu Thr Glu Asp Pro Asp Leu Gln Val Ser 
305                 310                 315                 320 


Ala Met Gln His Gln Thr Val Leu Glu Leu Thr Glu Thr Gly Val Glu 
                325                 330                 335     


Ala Ala Ala Ala Ser Ala Ile Ser Val Ala Arg Thr Leu Leu Val Phe 
            340                 345                 350         


Glu Val Gln Gln Pro Phe Leu Phe Val Leu Trp Asp Gln Gln His Lys 
        355                 360                 365             


Phe Pro Val Phe Met Gly Arg Val Tyr Asp Pro Arg Ala 
    370                 375                 380     


<210>  4
<211>  381
<212>  PRT
<213>  Artificial Sequence

<220>
<223>  Synthetic Polypeptide

<400>  4

Gly Ser Phe Cys Pro Gly Pro Val Thr Leu Cys Ser Asp Leu Glu Ser 
1               5                   10                  15      


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


Lys Leu Tyr His Ala Phe Ser Ala Met Lys Lys Val Glu Thr Asn Met 
        35                  40                  45              


Ala Phe Ser Pro Phe Ser Ile Ala Ser Leu Leu Thr Gln Val Leu Leu 
    50                  55                  60                  


Gly Ala Gly Glu Asn Thr Lys Thr Asn Leu Glu Ser Ile Leu Ser Tyr 
65                  70                  75                  80  


Pro Lys Asp Phe Thr Cys Val His Gln Ala Leu Lys Gly Phe Thr Thr 
                85                  90                  95      


Lys Gly Val Thr Ser Val Ser Gln Ile Phe His Ser Pro Asp Leu Ala 
            100                 105                 110         


Ile Arg Asp Thr Phe Val Asn Ala Ser Arg Thr Leu Tyr Ser Ser Ser 
        115                 120                 125             


Pro Arg Val Leu Ser Asn Asn Ser Asp Ala Asn Leu Glu Leu Ile Asn 
    130                 135                 140                 


Thr Trp Val Ala Lys Asn Thr Asn Asn Lys Ile Ser Arg Leu Leu Asp 
145                 150                 155                 160 


Ser Leu Pro Ser Asp Thr Arg Leu Val Leu Leu Asn Ala Ile Tyr Leu 
                165                 170                 175     


Ser Ala Lys Trp Lys Thr Thr Phe Asp Pro Lys Lys Thr Arg Met Glu 
            180                 185                 190         


Pro Phe His Phe Lys Asn Ser Val Ile Lys Val Pro Met Met Asn Ser 
        195                 200                 205             


Lys Lys Tyr Pro Val Ala His Phe Ile Asp Gln Thr Leu Lys Ala Lys 
    210                 215                 220                 


Val Gly Gln Leu Gln Leu Ser His Asn Leu Ser Leu Val Ile Leu Val 
225                 230                 235                 240 


Pro Gln Asn Leu Lys His Arg Leu Glu Asp Met Glu Gln Ala Leu Ser 
                245                 250                 255     


Pro Ser Val Phe Lys Ala Ile Met Glu Lys Leu Glu Met Ser Lys Phe 
            260                 265                 270         


Gln Pro Thr Leu Leu Thr Leu Pro Arg Ile Lys Val Thr Thr Ser Gln 
        275                 280                 285             


Asp Met Leu Ser Ile Met Glu Lys Leu Glu Phe Phe Asp Phe Ser Tyr 
    290                 295                 300                 


Asp Leu Asn Leu Cys Gly Leu Thr Glu Asp Pro Asp Leu Gln Val Ser 
305                 310                 315                 320 


Ala Met Gln His Gln Thr Val Leu Glu Leu Thr Glu Thr Gly Val Glu 
                325                 330                 335     


Ala Ala Ala Ala Ser Ala Ile Ser Val Ala Arg Thr Leu Leu Val Phe 
            340                 345                 350         


Glu Val Gln Gln Pro Phe Leu Phe Val Leu Trp Asp Gln Gln His Lys 
        355                 360                 365             


Phe Pro Val Phe Met Gly Arg Val Tyr Asp Pro Arg Ala 
    370                 375                 380     


