
                                SEQUENCE LISTING

<110> Gitler, Aaron D.
      Elden, Andrew

<120> MODULATORS OF TDP-43 MEDIATED TOXICITY
  AND METHODS OF USE THEREOF FOR IDENTIFYING AGENTS HAVING
  EFFICACY FOR THE TREATMENT AND PREVENTION OF PROTEINOPATHIES
  

<130> 1070-P04713WO00

<140> PCT/US2010/028821   
<141> 2010-03-26  

<150> 61/163,703          
<151> 2009-03-26  

<160> 2

<170> FastSEQ for Windows Version 4.0

<210> 1
<211> 414
<212> PRT
<213> Human

<400> 1
Met Ser Glu Tyr Ile Arg Val Thr Glu Asp Glu Asn Asp Glu Pro Ile
 1               5                  10                  15      
Glu Ile Pro Ser Glu Asp Asp Gly Thr Val Leu Leu Ser Thr Val Thr
            20                  25                  30          
Ala Gln Phe Pro Gly Ala Cys Gly Leu Arg Tyr Arg Asn Pro Val Ser
        35                  40                  45              
Gln Cys Met Arg Gly Val Arg Leu Val Glu Gly Ile Leu His Ala Pro
    50                  55                  60                  
Asp Ala Gly Trp Gly Asn Leu Val Tyr Val Val Asn Tyr Pro Lys Asp
65                  70                  75                  80  
Asn Lys Arg Lys Met Asp Glu Thr Asp Ala Ser Ser Ala Val Lys Val
                85                  90                  95      
Lys Arg Ala Val Gln Lys Thr Ser Asp Leu Ile Val Leu Gly Leu Pro
            100                 105                 110         
Trp Lys Thr Thr Glu Gln Asp Leu Lys Glu Tyr Phe Ser Thr Phe Gly
        115                 120                 125             
Glu Val Leu Met Val Gln Val Lys Lys Asp Leu Lys Thr Gly His Ser
    130                 135                 140                 
Lys Gly Phe Gly Phe Val Arg Phe Thr Glu Tyr Glu Thr Gln Val Lys
145                 150                 155                 160 
Val Met Ser Gln Arg His Met Ile Asp Gly Arg Trp Cys Asp Cys Lys
                165                 170                 175     
Leu Pro Asn Ser Lys Gln Ser Gln Asp Glu Pro Leu Arg Ser Arg Lys
            180                 185                 190         
Val Phe Val Gly Arg Cys Thr Glu Asp Met Thr Glu Asp Glu Leu Arg
        195                 200                 205             
Glu Phe Phe Ser Gln Tyr Gly Asp Val Met Asp Val Phe Ile Pro Lys
    210                 215                 220                 
Pro Phe Arg Ala Phe Ala Phe Val Thr Phe Ala Asp Asp Gln Ile Ala
225                 230                 235                 240 
Gln Ser Leu Cys Gly Glu Asp Leu Ile Ile Lys Gly Ile Ser Val His
                245                 250                 255     
Ile Ser Asn Ala Glu Pro Lys His Asn Ser Asn Arg Gln Leu Glu Arg
            260                 265                 270         
Ser Gly Arg Phe Gly Gly Asn Pro Gly Gly Phe Gly Asn Gln Gly Gly
        275                 280                 285             
Phe Gly Asn Ser Arg Gly Gly Gly Ala Gly Leu Gly Asn Asn Gln Gly
    290                 295                 300                 
Ser Asn Met Gly Gly Gly Met Asn Phe Gly Ala Phe Ser Ile Asn Pro
305                 310                 315                 320 
Ala Met Met Ala Ala Ala Gln Ala Ala Leu Gln Ser Ser Trp Gly Met
                325                 330                 335     
Met Gly Met Leu Ala Ser Gln Gln Asn Gln Ser Gly Pro Ser Gly Asn
            340                 345                 350         
Asn Gln Asn Gln Gly Asn Met Gln Arg Glu Pro Asn Gln Ala Phe Gly
        355                 360                 365             
Ser Gly Asn Asn Ser Tyr Ser Gly Ser Asn Ser Gly Ala Ala Ile Gly
    370                 375                 380                 
Trp Gly Ser Ala Ser Asn Ala Gly Ser Gly Ser Gly Phe Asn Gly Gly
385                 390                 395                 400 
Phe Gly Ser Ser Met Asp Ser Lys Ser Ser Gly Trp Gly Met
                405                 410                 


<210> 2
<211> 4236
<212> DNA
<213> Human

<400> 2
ggtgggcggg gggaggaggc ggccctagcg ccattttgtg ggagcgaagc ggtggctggg 60
ctgcgcttgg gtccgtcgct gcttcggtgt ccctgtcggg cttcccagca gcggcctagc 120
gggaaaagta aaagatgtct gaatatattc gggtaaccga agatgagaac gatgagccca 180
ttgaaatacc atcggaagac gatgggacgg tgctgctctc cacggttaca gcccagtttc 240
caggggcgtg tgggcttcgc tacaggaatc cagtgtctca gtgtatgaga ggtgtccggc 300
tggtagaagg aattctgcat gccccagatg ctggctgggg aaatctggtg tatgttgtca 360
actatccaaa agataacaaa agaaaaatgg atgagacaga tgcttcatca gcagtgaaag 420
tgaaaagagc agtccagaaa acatccgatt taatagtgtt gggtctccca tggaaaacaa 480
ccgaacagga cctgaaagag tattttagta cctttggaga agttcttatg gtgcaggtca 540
agaaagatct taagactggt cattcaaagg ggtttggctt tgttcgtttt acggaatatg 600
aaacacaagt gaaagtaatg tcacagcgac atatgataga tggacgatgg tgtgactgca 660
aacttcctaa ttctaagcaa agccaagatg agcctttgag aagcagaaaa gtgtttgtgg 720
ggcgctgtac agaggacatg actgaggatg agctgcggga gttcttctct cagtacgggg 780
atgtgatgga tgtcttcatc cccaagccat tcagggcctt tgcctttgtt acatttgcag 840
atgatcagat tgcgcagtct ctttgtggag aggacttgat cattaaagga atcagcgttc 900
atatatccaa tgccgaacct aagcacaata gcaatagaca gttagaaaga agtggaagat 960
ttggtggtaa tccaggtggc tttgggaatc agggtggatt tggtaatagc agagggggtg 1020
gagctggttt gggaaacaat caaggtagta atatgggtgg tgggatgaac tttggtgcgt 1080
tcagcattaa tccagccatg atggctgccg cccaggcagc actacagagc agttggggta 1140
tgatgggcat gttagccagc cagcagaacc agtcaggccc atcgggtaat aaccaaaacc 1200
aaggcaacat gcagagggag ccaaaccagg ccttcggttc tggaaataac tcttatagtg 1260
gctctaattc tggtgcagca attggttggg gatcagcatc caatgcaggg tcgggcagtg 1320
gttttaatgg aggctttggc tcaagcatgg attctaagtc ttctggctgg ggaatgtaga 1380
cagtggggtt gtggttggtt ggtatagaat ggtgggaatt caaatttttc taaactcatg 1440
gtaagtatat tgtaaaatac atatgtacta agaattttca aaattggttt gttcagtgtg 1500
gagtatattc agcagtattt ttgacatttt tctttagaaa aaggaagagc taaaggaatt 1560
ttataagttt tgttacatga aaggttgaaa tattgagtgg ttgaaagtga actgctgttt 1620
gcctgattgg taaaccaaca cactacaatt gatatcaaaa ggtttctcct gtaatatttt 1680
atccctggac ttgtcaagtg aattctttgc atgttcaaaa cggaaaccat tgattagaac 1740
tacattcttt accccttgtt ttaatttgaa ccccaccata tggatttttt tccttaagaa 1800
aatctccttt taggagatca tggtgtcaca gtgtttggtt cttttgtttt gttttttaac 1860
acttgtctcc cctcatacac aaaagtacaa tatgaagcct tcatttaatc tctgcagttc 1920
atctcatttc aaatgtttat ggaagaagca cttcattgaa agtagtgctg taaatattct 1980
gccataggaa tactgtctac atgctttctc attcaagaat tcgtcatcac gcatcacagg 2040
ccgcgtcttt gacggtgggt gtcccatttt tatccgctac tctttatttc atggagtcgt 2100
atcaacgcta tgaacgcaag gctgtgatat ggaaccagaa ggctgtctga acttttgaaa 2160
ccttgtgtgg gattgatggt ggtgccgagg catgaaaggc tagtatgagc gagaaaagga 2220
gagagcgcgt gcagagactt ggtggtgcat aatggatatt ttttaacttg gcgagatgtg 2280
tctctcaatc ctgtggcttt ggtgagagag tgtgcagaga gcaatgatag caaataatgt 2340
acgaatgttt tttgcattca aaggacatcc acatctgttg gaagactttt aagtgagttt 2400
ttgttcttag ataacccaca ttagatgaat gtgttaagtg aaatgatact tgtactcccc 2460
ctaccccttt gtcaactgct gtgaatgctg tatggtgtgt gttctcttct gttactgata 2520
tgtaagtgtg gcaatgtgaa ctgaagctga tgggctgaga acatggactg agcttgtggt 2580
gtgctttgca ggaggacttg aagcagagtt caccagtgag ctcaggtgtc tcaaagaagg 2640
gtggaagttc taatgtctgt tagctaccca taagaatgct gtttgctgca gttctgtgtc 2700
ctgtgcttgg atgcttttta taagagttgt cattgttgga aattcttaaa taaaactgat 2760
ttaaataata tgtgtctttg ttttgcagcc ctgaatgcaa agaattcata gcagttaatt 2820
cccctttttt gacccttttg agatggaact ttcataaagt ttcttggcag tagtttattt 2880
tgcttcaaat aaacttattt gaaaagttgt ctcaagtcaa atggattcat cacctgtcat 2940
gcattgacac ctgataccca gacttaattg gtatttgttc ttgcattggc caaagtgaaa 3000
attttttttt ttcttttgaa atctagtttt gaataagtct gggtgaccgc acctaaaatg 3060
gtaagcagta ccctccggct ttttcttagt gcctctgtgc atttgggtga tgttctattt 3120
acatggcctg tgtaaatctc cattgggaag tcatgccttc taaaaagatt cttatttggg 3180
ggagtgggca aaatgttgat tattttctaa tgctttgtag caaagcatat caattgaaaa 3240
gggaatatca gcaccttcct agtttgggat ttgaaaagtg gaattaattg cagtagggat 3300
aaagtagaag aaaccacaaa ttatcttgtg cctgaaatcc attaagaggc ctgatagctt 3360
taagaattag ggtgggttgt ctgtctggaa gtgttaagtg gaatgggctt tgtcctccag 3420
gaggtggggg aatgtggtaa cattgaatac agttgaataa aatcgcttac aaaactcaca 3480
ctctcacaat gcattgttaa gtatgtaaaa gcaataacat tgattctctg ttgtactttt 3540
ttgtaactaa ttctgtgaga gttgagctca ttttctagtt ggaagaatgt gatatttgtt 3600
gtgttggtag tttacctaat gcccttacct aattagatta tgataaatag gtttgtcatt 3660
ttgcaagtta cataaacatt tatcaatgaa gtcatccttt agacttgtaa tcgccacatt 3720
gtttcattat tcagtttcct ctgtaaaggg atcttgagtt gttttaattt tttttttctg 3780
catctgaatc tgcatgattt ccaaaccctg taccatctga attttgcatt ttagcacttg 3840
cactattact cagcagcagt aacatggtaa cacttaaaat ggtactcggg gacctccaaa 3900
gactaaactg acaagccttc aaggagccca ggggtaagtt aacttgtcaa cggcatggtt 3960
taatcccttc tttacacttg tgtaaatttc agttactggt catagaaggc tttcaatgtt 4020
gagtggcctt ttattaacat gtttatggta ctgcatagat acgggtattt attttaccct 4080
aagaagattt tgaagtttaa aagtacttaa actatttggc aaagatttgt ttttaaaaat 4140
ctatttggtc aatctaaatg cattcattct aaaaaatttt ttgaaccaga taaataaaat 4200
ttttttttga caccacaaaa aaaaaaaaaa aaaaaa                           4236
