                         SEQUENCE LISTING

<110>  Stichting Radboud universitair medisch centrum
 
<120>  Allele-specific silencing therapy for DFNA21 using antisense 
       oligonucleotides

<130>  P6102912PCT

<150>  EP21181836.4
<151>  2021-06-25

<160>  21    

<170>  PatentIn version 3.5

<210>  1
<211>  3195
<212>  DNA
<213>  Homo sapiens

<400>  1
atgttggtag gatcccagtc tttttcgcct ggagggccca atgggatcat tagaagccag       60

tcctttgcgg gtttcagcgg cctccaggaa aggcgatcca ggtgtaactc cttcattgaa      120

aattcctccg ctctcaagaa gcctcaggcc aaactgaaga aaatgcacaa tttaggccac      180

aaaaacaaca atccccccaa agagcctcag cctaaaaggg tggaagaagt ctacagggcc      240

ttgaaaaatg gacttgatga atatctggag gttcaccaga cggagctgga caagttgaca      300

gctcagttaa aagatatgaa aagaaactct cgcctgggtg tactgtatga cctagacaag      360

caaattaaaa caattgaaag atacatgaga cgcctggagt ttcatataag taaggtagat      420

gaactctatg aagcttattg tatccagcga cgcctccagg atggtgccag caaaatgaag      480

caagccttcg caacatcccc tgccagcaaa gctgcccggg agagtctgac agagatcaat      540

cggagcttca aggagtacac agagaatatg tgcaccattg aagtggagct agagaatctg      600

ctgggagaat tctccatcaa gatgaaaggt ctggctggct ttgcacgcct ctgtcctgga      660

gatcaatatg aaattttcat gaagtatggc cggcagcggt ggaaactgaa aggcaaaata      720

gaagtaaatg gcaagcagag ctgggatgga gaagaaacag tttttctgcc cctgatagtt      780

gggttcatct ccatcaaggt cacggagctc aaagggctag caactcacat cctggtaggt      840

agcgtgacct gtgagaccaa agagctgttt gcagcccgac ctcaggtagt ggctgtcgac      900

atcaatgacc ttggtaccat caaactgaac ctggaaatca cctggtatcc atttgacgtg      960

gaggacatga ccgcatcctc aggcgctggg aacaaggcag cagcccttca gaggagaatg     1020

tccatgtaca gccagggtac cccggaaacg cccaccttca aagaccactc cttctttagg     1080

tggctgcatc cttccccaga caagcccagg cggctgtctg tcttgagtgc cttgcaagac     1140

actttctttg ccaagctgca ccgcagccgc tccttcagtg acctgccctc cctcaggccg     1200

agtcccaagg ccgtgctaga gctctattca aatctacctg atgacatctt tgaaaatgga     1260

aaggcagccg aggagaaaat gccactgtcg ctcagcttca gtgacctgcc caacggggac     1320

tgcgccctca cctcccactc aacaggctcc ccttccaact caacaaatcc agaaattacc     1380

atcacccctg cggagtttaa cctcagcagc ttggcctccc agaatgaggg tatggatgac     1440

accagctcag catcttccag gaactccctg ggagaaggcc aagagccaaa gtcacacctg     1500

aaggaggaag acccagagga gcccagaaaa cctgcctcgg ccccatctga ggcttgccgc     1560

cgacagtcct caggtgctgg ggctgagcac ctgttccttg agaatgatgt tgcagaagca     1620

cttctgcaag agtctgagga ggcctctgag ctcaagcctg tggaactgga cacttcggaa     1680

ggaaacatca caaagaggct cacatctgca gaggtgccaa tggccacaga caggctgctc     1740

tctgagggtt ctgttggtgg agaatctgaa ggctgcagat cctttctaga tggaagctta     1800

gaggatgctt ttaatgggct tttacttgca ttagaaccac ataaagagca gtataaagag     1860

tttcaggatc tgaaccaaga agtcatgaat ttggatgata ttctaaaatg caagccagca     1920

gtaagccgca gcaggtcttc cagtttaagt ctcacagttg aaagtgcttt agaaagcttt     1980

gatttcctga acacctctga ttttgacgag gaggaggatg gtgatgaggt ttgtaatgtt     2040

ggcggaggtg ctgactcagt attttcagac actgagactg agaaacacag ttacaggtcg     2100

gttcacccag aagccagggg gcatctcagt gaagcgctca ctgaagacac aggagttggg     2160

accagtgtgg caggaagtcc tctcccactg accacaggca acgagagcct ggacatcacc     2220

atcgtcaggc acctccagta ctgcacccaa ctcgtgcagc aaattgtttt ctcaagcaaa     2280

accccatttg tggcaagaag tctcttagag aagctttcta ggcagatcca agtgatggag     2340

aaactcgcag ctgtcagtga tgagaacata ggaaatatca gttctgttgt ggaagccata     2400

ccagaatttc acaaaaagct gtctttgctg tcattctgga ccaagtgctg cagccctgtt     2460

ggtgtctacc acagcccagc ggacagagtg atgaagcagc tggaggccag ctttgccaga     2520

actgtcaaca aagaatatcc aggacttgca gacccagtgt ttcgaaccct ggtgtcccaa     2580

attctggacc gggctgagcc tctgctttcc tccagcctgt cctcggaagt cgtcactgtt     2640

ttccagtatt acagttactt caccagccac ggcgtcagtg acctggagag ttacctgagc     2700

cagctggcca ggcaagtttc catggttcag actctgcaat cactaagaga tgaaaaactg     2760

ctacaaacca tgagtgacct tgctcccagc aacctcctgg cccagcagga agtactcagg     2820

actctggctc tgctattaac cagagaggac aacgaagtta gcgaggctgt gacgctttac     2880

ttggcagcag cctccaaaaa tcagcatttc agggaaaagg ccttgctcta ttactgtgaa     2940

gcactaacaa agacaaacct ccagctccag aaagcagctt gcctggctct gaaaatcctt     3000

gaggctactg aaagcattaa aatgctggtg acattgtgtc aatctgatac tgaagaaatc     3060

agaaatgtgg cctcagaaac cctcttgtct ctgggagaag atgggcggct ggcatatgaa     3120

caattggaca aatttcctcg agactgtgtt aaagtcggag gtcgtcatgg aactgaagtt     3180

gccacagcct tttaa                                                      3195


<210>  2
<211>  60
<212>  DNA
<213>  Homo sapiens

<400>  2
gaactggaca cttcggaagg aaacatcaca aagaggctca catctgcaga ggtgccaatg       60


<210>  3
<211>  50
<212>  DNA
<213>  Homo sapiens

<400>  3
ggacacttcg gaaggaaaca tcacaaagag gctcacatct gcagaggtgc                  50


<210>  4
<211>  40
<212>  DNA
<213>  Homo sapiens

<400>  4
cttcggaagg aaacatcaca aagaggctca catctgcaga                             40


<210>  5
<211>  20
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  AON-1

<400>  5
gagcctcttt gtgatgtttc                                                   20


<210>  6
<211>  20
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  AON-2

<400>  6
tgtgagcctc tttgtgatgt                                                   20


<210>  7
<211>  20
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  AON-3

<400>  7
agatgtgagc ctctttgtga                                                   20


<210>  8
<211>  19
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  AON-4

<400>  8
tgtgagcctc tttgtgatg                                                    19


<210>  9
<211>  18
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  AON-5

<400>  9
tgtgagcctc tttgtgat                                                     18


<210>  10
<211>  20
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  AON6

<400>  10
gagcctcttt gtgauguuuc                                                   20


<210>  11
<211>  20
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  AON-7

<400>  11
augugagcct ctttgugaug                                                   20


<210>  12
<211>  1830
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  cDNA sequence encoding an N-terminally FLAG-tagged mutant RIPOR2

<400>  12
atggactaca aagacgatga cgacaagctt gcggccatca caagtttgta caaaaaagca       60

ggcttcatgt tggtaggatc ccagtctttt tcgcctggag ggcccaatgg gatcattaga      120

agccagtcct ttgcgggttt cagcggcctc caggaaaggc gatccaggtg taactccttc      180

attgaaaatt cctccgctct caagaagcct caggccaaac tgaagaaaat gcacaattta      240

ggccacaaaa acaacaatcc ccccaaagag cctcagccta aaagggtgga agaagtctac      300

agggccttga aaaatggact tgatgaatat ctggaggttc accagacgga gctggacaag      360

ttgacagctc agttaaaaga tatgaaaaga aactctcgcc tgggtgtact gtatgaccta      420

gacaagcaaa ttaaaacaat tgaaagatac atgagacgcc tggagtttca tataagtaag      480

gtagatgaac tctatgaagc ttattgtatc cagcgacgcc tccaggatgg tgccagcaaa      540

atgaagcaag ccttcgcaac atcccctgcc agcaaagctg cccgggagag tctgacagag      600

atcaatcgga gcttcaagga gtacacagag aatatgtgca ccattgaagt ggagctagag      660

aatctgctgg gagaattctc catcaagatg aaaggtctgg ctggctttgc acgcctctgt      720

cctggagatc aatatgaaat tttcatgaag tatggccggc agcggtggaa actgaaaggc      780

aaaatagaag taaatggcaa gcagagctgg gatggagaag aaacagtttt tctgcccctg      840

atagttgggt tcatctccat caaggtcacg gagctcaaag ggctagcaac tcacatcctg      900

gtaggtagcg tgacctgtga gaccaaagag ctgtttgcag cccgacctca ggtagtggct      960

gtcgacatca atgaccttgg taccatcaaa ctgaacctgg aaatcacctg gtatccattt     1020

gacgtggagg acatgaccgc atcctcaggc gctgggaaca aggcagcagc ccttcagagg     1080

agaatgtcca tgtacagcca gggtaccccg gaaacgccca ccttcaaaga ccactccttc     1140

ttttcaaatc tacctgatga catctttgaa aatggaaagg cagccgagga gaaaatgcca     1200

ctgtcgctca gcttcagtga cctgcccaac ggggactgcg ccctcacctc ccactcaaca     1260

ggctcccctt ccaactcaac aaatccagaa attaccatca cccctgcgga gtttaacctc     1320

agcagcttgg cctcccagaa tgagggtatg gatgacacca gctcagcatc ttccaggaac     1380

tccctgggag aaggccaaga gccaaagtca cacctgaagg aggaagaccc agaggagccc     1440

agaaaacctg cctcggcccc atctgaggct tgccgccgac agtcctcagg tgctggggct     1500

gagcacctgt tccttgagaa tgatgttgca gaagcacttc tgcaagagtc tgaggaggcc     1560

tctgagctca agcctgtgga actggacact tcggaaggaa acatcacaaa gaggctcaca     1620

tctgcagagg tgccaatggc cacagacagg ctgctctctg agggttctgt tggtggagaa     1680

tctgaaggct gcagatcctt tctagatgga agcttagagg atgcttttaa tgggctttta     1740

cttgcattag aaccacataa agagcagtat aaagagtttc aggatctgaa ccaagaagtc     1800

atgaatttgg atgatattct aaaaaagtag                                      1830


<210>  13
<211>  21
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  primer

<400>  13
ggaaggaaac atcacaaaga g                                                 21


<210>  14
<211>  18
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  primer

<400>  14
gcagccttca gattctcc                                                     18


<210>  15
<211>  20
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  primer

<400>  15
agagtggtgc tgaggattgg                                                   20


<210>  16
<211>  20
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  primer

<400>  16
ccctcatgct ctagcgtgtc                                                   20


<210>  17
<211>  20
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  control AON

<400>  17
auagtcttcc tgtuguggcu                                                   20


<210>  18
<211>  46
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  Fig 1B.

<400>  18
aaggaaacat cacaaagcag ctggtcaaga ggctcacatc tgcaga                      46


<210>  19
<211>  3123
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  cDNA sequences encoding a C-terminally HA-tagged mutant RIPOR2

<400>  19
atgttggtag gatcccagtc tttttcgcct ggagggccca atgggatcat tagaagccag       60

tcctttgcgg gtttcagcgg cctccaggaa aggcgatcca ggtgtaactc cttcattgaa      120

aattcctccg ctctcaagaa gcctcaggcc aaactgaaga aaatgcacaa tttaggccac      180

aaaaacaaca atccccccaa agagcctcag cctaaaaggg tggaagaagt ctacagggcc      240

ttgaaaaatg gacttgatga atatctggag gttcaccaga cggagctgga caagttgaca      300

gctcagttaa aagatatgaa aagaaactct cgcctgggtg tactgtatga cctagacaag      360

caaattaaaa caattgaaag atacatgaga cgcctggagt ttcatataag taaggtagat      420

gaactctatg aagcttattg tatccagcga cgcctccagg atggtgccag caaaatgaag      480

caagccttcg caacatcccc tgccagcaaa gctgcccggg agagtctgac agagatcaat      540

cggagcttca aggagtacac agagaatatg tgcaccattg aagtggagct agagaatctg      600

ctgggagaat tctccatcaa gatgaaaggt ctggctggct ttgcacgcct ctgtcctgga      660

gatcaatatg aaattttcat gaagtatggc cggcagcggt ggaaactgaa aggcaaaata      720

gaagtaaatg gcaagcagag ctgggatgga gaagaaacag tttttctgcc cctgatagtt      780

gggttcatct ccatcaaggt cacggagctc aaagggctag caactcacat cctggtaggt      840

agcgtgacct gtgagaccaa agagctgttt gcagcccgac ctcaggtagt ggctgtcgac      900

atcaatgacc ttggtaccat caaactgaac ctggaaatca cctggtatcc atttgacgtg      960

gaggacatga ccgcatcctc aggcgctggg aacaaggcag cagcccttca gaggagaatg     1020

tccatgtaca gccagggtac cccggaaacg cccaccttca aagaccactc cttcttttca     1080

aatctacctg atgacatctt tgaaaatgga aaggcagccg aggagaaaat gccactgtcg     1140

ctcagcttca gtgacctgcc caacggggac tgcgccctca cctcccactc aacaggctcc     1200

ccttccaact caacaaatcc agaaattacc atcacccctg cggagtttaa cctcagcagc     1260

ttggcctccc agaatgaggg tatggatgac accagctcag catcttccag gaactccctg     1320

ggagaaggcc aagagccaaa gtcacacctg aaggaggaag acccagagga gcccagaaaa     1380

cctgcctcgg ccccatctga ggcttgccgc cgacagtcct caggtgctgg ggctgagcac     1440

ctgttccttg agaatgatgt tgcagaagca cttctgcaag agtctgagga ggcctctgag     1500

ctcaagcctg tggaactgga cacttcggaa ggaaacatca caaagaggct cacatctgca     1560

gaggtgccaa tggccacaga caggctgctc tctgagggtt ctgttggtgg agaatctgaa     1620

ggctgcagat cctttctaga tggaagctta gaggatgctt ttaatgggct tttacttgca     1680

ttagaaccac ataaagagca gtataaagag tttcaggatc tgaaccaaga agtcatgaat     1740

ttggatgata ttctaaaatg caagccagca gtaagccgca gcaggtcttc cagtttaagt     1800

ctcacagttg aaagtgcttt agaaagcttt gatttcctga acacctctga ttttgacgag     1860

gaggaggatg gtgatgaggt ttgtaatgtt ggcggaggtg ctgactcagt attttcagac     1920

actgagactg agaaacacag ttacaggtcg gttcacccag aagccagggg gcatctcagt     1980

gaagcgctca ctgaagacac aggagttggg accagtgtgg caggaagtcc tctcccactg     2040

accacaggca acgagagcct ggacatcacc atcgtcaggc acctccagta ctgcacccaa     2100

ctcgtgcagc aaattgtttt ctcaagcaaa accccatttg tggcaagaag tctcttagag     2160

aagctttcta ggcagatcca agtgatggag aaactcgcag ctgtcagtga tgagaacata     2220

ggaaatatca gttctgttgt ggaagccata ccagaatttc acaaaaagct gtctttgctg     2280

tcattctgga ccaagtgctg cagccctgtt ggtgtctacc acagcccagc ggacagagtg     2340

atgaagcagc tggaggccag ctttgccaga actgtcaaca aagaatatcc aggacttgca     2400

gacccagtgt ttcgaaccct ggtgtcccaa attctggacc gggctgagcc tctgctttcc     2460

tccagcctgt cctcggaagt cgtcactgtt ttccagtatt acagttactt caccagccac     2520

ggcgtcagtg acctggagag ttacctgagc cagctggcca ggcaagtttc catggttcag     2580

actctgcaat cactaagaga tgaaaaactg ctacaaacca tgagtgacct tgctcccagc     2640

aacctcctgg cccagcagga agtactcagg actctggctc tgctattaac cagagaggac     2700

aacgaagtta gtgaggctgt gacgctttac ttggcagcag cctccaaaaa tcagcatttc     2760

agggaaaagg ccttgctcta ttactgtgaa gcactaacaa agacaaacct ccagctccag     2820

aaagcagctt gcctggctct gaaaatcctt gaggctactg aaagcattaa aatgctggtg     2880

acattgtgtc aatctgatac tgaagaaatc agaaatgtgg cctcagaaac cctcttgtct     2940

ctgggagaag atgggcggct ggcatatgaa caattggaca aatttcctcg agactgtgtt     3000

aaagtcggag gtcgtcatgg aactgaagtt gccacagcct ttgacccagc tttcttgtac     3060

aaagtggttc gatctagaat ggctagctat ccgtatgatg ttcctgatta tgctagcctc     3120

tag                                                                   3123


<210>  20
<211>  3135
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  cDNA sequences encoding a C-terminally HA-tagged wildtype RIPOR2

<400>  20
atgttggtag gatcccagtc tttttcgcct ggagggccca atgggatcat tagaagccag       60

tcctttgcgg gtttcagcgg cctccaggaa aggcgatcca ggtgtaactc cttcattgaa      120

aattcctccg ctctcaagaa gcctcaggcc aaactgaaga aaatgcacaa tttaggccac      180

aaaaacaaca atccccccaa agagcctcag cctaaaaggg tggaagaagt ctacagggcc      240

ttgaaaaatg gacttgatga atatctggag gttcaccaga cggagctgga caagttgaca      300

gctcagttaa aagatatgaa aagaaactct cgcctgggtg tactgtatga cctagacaag      360

caaattaaaa caattgaaag atacatgaga cgcctggagt ttcatataag taaggtagat      420

gaactctatg aagcttattg tatccagcga cgcctccagg atggtgccag caaaatgaag      480

caagccttcg caacatcccc tgccagcaaa gctgcccggg agagtctgac agagatcaat      540

cggagcttca aggagtacac agagaatatg tgcaccattg aagtggagct agagaatctg      600

ctgggagaat tctccatcaa gatgaaaggt ctggctggct ttgcacgcct ctgtcctgga      660

gatcaatatg aaattttcat gaagtatggc cggcagcggt ggaaactgaa aggcaaaata      720

gaagtaaatg gcaagcagag ctgggatgga gaagaaacag tttttctgcc cctgatagtt      780

gggttcatct ccatcaaggt cacggagctc aaagggctag caactcacat cctggtaggt      840

agcgtgacct gtgagaccaa agagctgttt gcagcccgac ctcaggtagt ggctgtcgac      900

atcaatgacc ttggtaccat caaactgaac ctggaaatca cctggtatcc atttgacgtg      960

gaggacatga ccgcatcctc aggcgctggg aacaaggcag cagcccttca gaggagaatg     1020

tccatgtaca gccagggtac cccggaaacg cccaccttca aagaccactc cttcttttca     1080

aatctacctg atgacatctt tgaaaatgga aaggcagccg aggagaaaat gccactgtcg     1140

ctcagcttca gtgacctgcc caacggggac tgcgccctca cctcccactc aacaggctcc     1200

ccttccaact caacaaatcc agaaattacc atcacccctg cggagtttaa cctcagcagc     1260

ttggcctccc agaatgaggg tatggatgac accagctcag catcttccag gaactccctg     1320

ggagaaggcc aagagccaaa gtcacacctg aaggaggaag acccagagga gcccagaaaa     1380

cctgcctcgg ccccatctga ggcttgccgc cgacagtcct caggtgctgg ggctgagcac     1440

ctgttccttg agaatgatgt tgcagaagca cttctgcaag agtctgagga ggcctctgag     1500

ctcaagcctg tggaactgga cacttcggaa ggaaacatca caaagcagct ggtcaagagg     1560

ctcacatctg cagaggtgcc aatggccaca gacaggctgc tctctgaggg ttctgttggt     1620

ggagaatctg aaggctgcag atcctttcta gatggaagct tagaggatgc ttttaatggg     1680

cttttacttg cattagaacc acataaagag cagtataaag agtttcagga tctgaaccaa     1740

gaagtcatga atttggatga tattctaaaa tgcaagccag cagtaagccg cagcaggtct     1800

tccagtttaa gtctcacagt tgaaagtgct ttagaaagct ttgatttcct gaacacctct     1860

gattttgacg aggaggagga tggtgatgag gtttgtaatg ttggcggagg tgctgactca     1920

gtattttcag acactgagac tgagaaacac agttacaggt cggttcaccc agaagccagg     1980

gggcatctca gtgaagcgct cactgaagac acaggagttg ggaccagtgt ggcaggaagt     2040

cctctcccac tgaccacagg caacgagagc ctggacatca ccatcgtcag gcacctccag     2100

tactgcaccc aactcgtgca gcaaattgtt ttctcaagca aaaccccatt tgtggcaaga     2160

agtctcttag agaagctttc taggcagatc caagtgatgg agaaactcgc agctgtcagt     2220

gatgagaaca taggaaatat cagttctgtt gtggaagcca taccagaatt tcacaaaaag     2280

ctgtctttgc tgtcattctg gaccaagtgc tgcagccctg ttggtgtcta ccacagccca     2340

gcggacagag tgatgaagca gctggaggcc agctttgcca gaactgtcaa caaagaatat     2400

ccaggacttg cagacccagt gtttcgaacc ctggtgtccc aaattctgga ccgggctgag     2460

cctctgcttt cctccagcct gtcctcggaa gtcgtcactg ttttccagta ttacagttac     2520

ttcaccagcc acggcgtcag tgacctggag agttacctga gccagctggc caggcaagtt     2580

tccatggttc agactctgca atcactaaga gatgaaaaac tgctacaaac catgagtgac     2640

cttgctccca gcaacctcct ggcccagcag gaagtactca ggactctggc tctgctatta     2700

accagagagg acaacgaagt tagtgaggct gtgacgcttt acttggcagc agcctccaaa     2760

aatcagcatt tcagggaaaa ggccttgctc tattactgtg aagcactaac aaagacaaac     2820

ctccagctcc agaaagcagc ttgcctggct ctgaaaatcc ttgaggctac tgaaagcatt     2880

aaaatgctgg tgacattgtg tcaatctgat actgaagaaa tcagaaatgt ggcctcagaa     2940

accctcttgt ctctgggaga agatgggcgg ctggcatatg aacaattgga caaatttcct     3000

cgagactgtg ttaaagtcgg aggtcgtcat ggaactgaag ttgccacagc ctttgaccca     3060

gctttcttgt acaaagtggt tcgatctaga atggctagct atccgtatga tgttcctgat     3120

tatgctagcc tctag                                                      3135


<210>  21
<211>  20
<212>  DNA
<213>  Artificial Sequence

<220>
<223>  AON6-8oxoG


<220>
<221>  misc_feature
<222>  (16)..(16)
<223>  n is an g modified 8-oxoguanine base

<400>  21
gagcctcttt gtgaunuuuc                                                   20


