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All mouse models of auditory system disease with phenotypic similarity to the human disease
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| Disease Term | Allelic Composition | Genetic Background | Reference | Phenotypes | ||||
  | autosomal dominant auditory neuropathy 1 | Tg(CAG-Diap3)924Lesp/0 | FVB/NJ-Tg(CAG-Diap3)924Lesp | J:193237 | View | |||
| autosomal dominant auditory neuropathy 1 | Tg(CAG-Diaph3)771Lesp/0 | FVB/NJ-Tg(CAG-Diaph3)771Lesp | J:193237 | View | ||||
| autosomal dominant nonsyndromic deafness 9 | Cochtm1Mrtn/Cochtm1Mrtn | CBACa.129S4-Cochtm1Mrtn | J:140594, J:167669 | View | ||||
| autosomal dominant nonsyndromic deafness 12 | Tectatm2Gpr/Tecta+ | involves: 129S/SvEv * C57BL/6J | J:101691 | View | ||||
| autosomal dominant nonsyndromic deafness 12 | Tectatm3.1Gpr/Tecta+ | involves: 129S/SvEv | J:203482 | View | ||||
| autosomal dominant nonsyndromic deafness 12 | Tectatm4.1Gpr/Tecta+ | involves: 129S/SvEv | J:203482 | View | ||||
| autosomal dominant nonsyndromic deafness 12 | Tectatm5.1Gpr/Tecta+ | involves: 129S/SvEv | J:203482 | View | ||||
| autosomal dominant nonsyndromic deafness 13 | Col11a2tm1Mne/Col11a2tm1Mne | FVB.129-Col11a2tm1Mne | J:71948 | View | ||||
| autosomal dominant nonsyndromic deafness 22 | Myo6em1Bcgen/Myo6+ | involves: C57BL/6J * CBA/CaJ | J:288210 | View | ||||
| autosomal dominant nonsyndromic deafness 22 | Myo6sv/Myo6sv | involves: B10.HA/(33NX)Sn * C57BL/6J | J:29898 | View | ||||
| autosomal dominant nonsyndromic deafness 25 | Slc17a8tm1Selm/Slc17a8tm1Selm | involves: 129/Sv * C57BL/6 | J:139493 | View | ||||
| autosomal dominant nonsyndromic deafness 36 | Tmc1dn/Tmc1dn | involves: STOCK Grhl3ct * M. m. molossinus | J:22445 | View | ||||
| autosomal dominant nonsyndromic deafness 36 | Tmc1dn/Tmc1dn | STOCK Grhl3ct/J | J:236 | View | ||||
| autosomal dominant nonsyndromic deafness 36 | Tmc1Mhdabth/Tmc1+ | C3HeB/FeJ-Tmc1Mhdabth/Ieg | J:86685 | View | ||||
| autosomal dominant nonsyndromic deafness 41 | P2rx2em1Xzl/P2rx2+ | CBA/J-P2rx2em1Xzl | J:315007 | View | ||||
| autosomal dominant nonsyndromic deafness 67 | Osbpl2em1Cya/Osbpl2em1Cya | C57BL/6-Osbpl2em1Cya | J:324115 | View | ||||
| autosomal recessive nonsyndromic deafness 1A | Del(14Gjb6-Cryl1)1Lmon/Del(14Gjb6-Cryl1)1Lmon | C57BL/6JOlaHsd-Del(14Gjb6-Cryl1)1Lmon/Lmon | J:345081 | View | ||||
| autosomal recessive nonsyndromic deafness 1A |
Gjb2tm1Ugds/Gjb2tm1Ugds Tg(Otog-cre)1Ugds/0 |
involves: 129P2/OlaHsd * C57BL/6 | J:77823 | View | ||||
| autosomal recessive nonsyndromic deafness 1A |
Gjb2tm1Ugds/Gjb2tm1Ugds Tg(Sox10-cre)1Wdr/0 |
involves: 129P2/OlaHsd * C57BL/6 * CBA | J:206835 | View | ||||
| autosomal recessive nonsyndromic deafness 1A | Gjb6tm1.1Fama/Gjb6tm1.1Fama | involves: 129P2/OlaHsd * C57BL/6 * SJL | J:166362 | View | ||||
| autosomal recessive nonsyndromic deafness 1A | Gjb6tm1Kwi/Gjb6tm1Kwi | involves: 129P2/OlaHsd * C57BL/6 | J:80917 | View | ||||
| autosomal recessive nonsyndromic deafness 2 | Myo7admbo2/Myo7admbo2 | involves: C57BL/6J | J:195666 | View | ||||
| autosomal recessive nonsyndromic deafness 3 | Myo15ash2-2J/Myo15ash2-2J | involves: STOCK Rb(16.17)7Bnr | J:69998 | View | ||||
| autosomal recessive nonsyndromic deafness 3 | Myo15atm1.1Jebd/Myo15atm1.1Jebd | involves: C57BL/6 * FVB/N | J:226820 | View | ||||
| autosomal recessive nonsyndromic deafness 7 | Tmc1baringo/Tmc1baringo | involves: C57BL/6 | J:181985 | View | ||||
| autosomal recessive nonsyndromic deafness 7 | Tmc1dn/Tmc1dn | involves: STOCK Grhl3ct * M. m. molossinus | J:22445 | View | ||||
| autosomal recessive nonsyndromic deafness 7 | Tmc1dn/Tmc1dn | STOCK Grhl3ct/J | J:236 | View | ||||
| autosomal recessive nonsyndromic deafness 7 | Tmc1Mhdabth/Tmc1+ | C3HeB/FeJ-Tmc1Mhdabth/Ieg | J:86685 | View | ||||
| autosomal recessive nonsyndromic deafness 7 | Tmc1nice/Tmc1nice | involves: C57BL/6 | J:181985 | View | ||||
| autosomal recessive nonsyndromic deafness 7 | Tmc1stitch/Tmc1stitch | involves: C57BL/6 | J:181985 | View | ||||
| autosomal recessive nonsyndromic deafness 9 | Otofdeaf5Jcs/Otofdeaf5Jcs | STOCK Otofdeaf5Jcs/Kjn | J:125960 | View | ||||
| autosomal recessive nonsyndromic deafness 9 | Otoftm1Ugds/Otoftm1Ugds | involves: 129S2/SvPas * C57BL/6 | J:116097 | View | ||||
| autosomal recessive nonsyndromic deafness 12 | Cdh2312J/Cdh2312J | involves: C57BL/6J | J:174758 | View | ||||
| autosomal recessive nonsyndromic deafness 12 | Cdh23Jera/Cdh23Jera | involves: C57BL/6 | J:174130 | View | ||||
| autosomal recessive nonsyndromic deafness 12 | Cdh23sals/Cdh23sals | involves: C57BL/6J | J:147149 | View | ||||
| autosomal recessive nonsyndromic deafness 16 | Strctm1Ugds/Strctm1Ugds | involves: 129S2/SvPas * BALB/c * C57BL/6 * C57BL/6J | J:141421 | View | ||||
| autosomal recessive nonsyndromic deafness 18A | Ush1cdfcr-2J/Ush1cdfcr-2J | B6;129S4 | J:85400 | View | ||||
| autosomal recessive nonsyndromic deafness 18A | Ush1cdfcr-4J/Ush1cdfcr-4J | 129P3/J-Ush1cdfcr-4J/J | J:278662 | View | ||||
| autosomal recessive nonsyndromic deafness 18A | Ush1cdfcr/Ush1cdfcr | involves: BALB/cByJ | J:85400 | View | ||||
| autosomal recessive nonsyndromic deafness 18B | Otogvbd/Otogvbd | involves: C3HeB/FeJ * C57BL/6J | J:234038 | View | ||||
| autosomal recessive nonsyndromic deafness 22 | Otoatm1Gpr/Otoatm1Gpr | involves: 129S/SvEv | J:192263 | View | ||||
| autosomal recessive nonsyndromic deafness 23 | Pcdh15roda/Pcdh15roda | BALB/c-Pcdh15roda | J:220658 | View | ||||
| autosomal recessive nonsyndromic deafness 28 | Triobptm1Tbf/Triobptm1Tbf | C57BL/6-Triobptm1Tbf | J:167947 | View | ||||
| autosomal recessive nonsyndromic deafness 29 | Cldn14tm1Tbf/Cldn14tm1Tbf | involves: 129S1/Sv * 129X1/SvJ * C57BL/6 | J:85071 | View | ||||
| autosomal recessive nonsyndromic deafness 30 | Myo3atm1.1Mckg/Myo3atm1.1Mckg | C57BL/6-Myo3atm1.1Mckg | J:166812 | View | ||||
| autosomal recessive nonsyndromic deafness 31 | Whrnwi/Whrnwi | involves: C57BL/6J * STOCK a Tyrp1b Myo5ad Oca2p Ednrbs | J:269, J:5037, J:5538, J:77939 | View | ||||
| autosomal recessive nonsyndromic deafness 32 | Cdc14aem1Tbf/Cdc14aem1Tbf | involves: C57BL/6J | J:257652 | View | ||||
| autosomal recessive nonsyndromic deafness 32 | Cdc14atm1a(EUCOMM)Hmgu/Cdc14atm1b(EUCOMM)Hmgu | involves: C57BL/6N * FVB | J:257652 | View | ||||
| autosomal recessive nonsyndromic deafness 32 | Cdc14atm1b(EUCOMM)Hmgu/Cdc14atm1b(EUCOMM)Hmgu | involves: C57BL/6N * FVB | J:257652 | View | ||||
| autosomal recessive nonsyndromic deafness 32 | Cdc14atm1d(EUCOMM)Hmgu/Cdc14atm1d(EUCOMM)Hmgu | involves: C57BL/6N * FVB | J:257652 | View | ||||
| autosomal recessive nonsyndromic deafness 37 | Myo6sv/Myo6sv | involves: B10.HA/(33NX)Sn * C57BL/6J | J:29898 | View | ||||
| autosomal recessive nonsyndromic deafness 39 | Hgftm1.1Tbf/Hgftm1.1Tbf | B6.Cg-Hgftm1.1Tbf/Tbf | J:289982 | View | ||||
| autosomal recessive nonsyndromic deafness 39 | Hgftm1Tbf/Hgftm1Tbf | B6.Cg-Hgftm1Tbf/Tbf | J:289982 | View | ||||
| autosomal recessive nonsyndromic deafness 42 | Ildr1Gt(D178D03)Wrst/Ildr1Gt(D178D03)Wrst | involves: 129S2/SvPas * Swiss Webster | J:217755 | View | ||||
| autosomal recessive nonsyndromic deafness 42 | Ildr1tm1(KOMP)Wtsi/Ildr1tm1(KOMP)Wtsi | involves: C57BL/6N | J:217755, J:221592 | View | ||||
| autosomal recessive nonsyndromic deafness 42 | Ildr1tm1.1Lwa/Ildr1tm1.1Lwa | involves: 129S6/SvEvTac * C57BL/6 | J:221594 | View | ||||
| autosomal recessive nonsyndromic deafness 48 | Cib2tm1.1Aela/Cib2tm1.1Aela | involves: BALB/c * C57BL/6 * C57BL/6N | J:262464 | View | ||||
| autosomal recessive nonsyndromic deafness 49 | Marveld2tm1.1Sria/Marveld2tm1.1Sria | involves: 129X1/SvJ * C57BL/6J | J:201580 | View | ||||
| autosomal recessive nonsyndromic deafness 49 | Marveld2tm1Sats/Marveld2tm1Sats | B6.Cg-Marveld2tm1Sats | J:261357 | View | ||||
| autosomal recessive nonsyndromic deafness 59 | Pjvktm1Ugds/Pjvktm1Ugds | involves: 129S2/SvPas * BALB/c * C57BL/6 | J:111260 | View | ||||
| autosomal recessive nonsyndromic deafness 63 | Tomtm1Btlr/Tomtm1Btlr | C57BL/6J-Tomtm1Btlr | J:137493 | View | ||||
| autosomal recessive nonsyndromic deafness 68 | S1pr2stdf/S1pr2stdf | involves: C57BL/6Brd * C57BL/6N | J:240361 | View | ||||
| autosomal recessive nonsyndromic deafness 68 | S1pr2tm1Ajml/S1pr2tm1Ajml | either: (involves: 129S5/SvEvBrd) or (involves: 129S5/SvEvBrd * C57BL/6) | J:231927 | View | ||||
| autosomal recessive nonsyndromic deafness 74 | Msrb3tm1Hyk/Msrb3tm1Hyk | involves: 129 * C57BL/6 | J:206322 | View | ||||
| autosomal recessive nonsyndromic deafness 79 | Tprnem1Pghu/Tprnem1Pghu | B6.Cg-Tprnem1Pghu | J:240563 | View | ||||
| autosomal recessive nonsyndromic deafness 88 | Elmod3em1Jili/Elmod3em1Jili | involves: C57BL/6 | J:293683 | View | ||||
| autosomal recessive nonsyndromic deafness 93 | Cabp2tm1b(KOMP)Mbp/Cabp2tm1b(KOMP)Mbp | involves: C57BL/6N * FVB/N | J:255028 | View | ||||
| X-linked deafness 4 | Smpxem1Jgao/Smpx+ | CBA/CaJ-Smpxem1Jgao | J:314284 | View | ||||
| X-linked deafness 4 | Smpxem1Jgao/Smpxem1Jgao | CBA/CaJ-Smpxem1Jgao | J:314284 | View | ||||
| X-linked deafness 4 | Smpxem1Jgao/Y | CBA/CaJ-Smpxem1Jgao | J:314284 | View | ||||
| X-linked deafness 4 | Smpxem2Jgao/Smpx+ | CBA/CaJ-Smpxem2Jgao | J:314284 | View | ||||
| X-linked deafness 4 | Smpxem2Jgao/Smpxem2Jgao | CBA/CaJ-Smpxem2Jgao | J:314284 | View | ||||
| X-linked deafness 4 | Smpxem2Jgao/Y | CBA/CaJ-Smpxem2Jgao | J:314284 | View | ||||
| | auditory system disease | Fscn2em1Fhan/Fscn2em1Fhan | C57BL/6J-Fscn2em1Fhan | J:274170 | View | |||
| autosomal dominant nonsyndromic deafness 4A | Ceacam16tm1Wzm/Ceacam16tm1Wzm | BALB/cJ-Ceacam16tm1Wzm | J:187537 | View | ||||
| autosomal dominant nonsyndromic deafness 25 | Trpv4tm1Msz/Trpv4tm1Msz | involves: 129X1/SvJ * C57BL/6 | J:104851 | View | ||||
| autosomal recessive nonsyndromic deafness | Atp6v1b1vtx/Atp6v1b1vtx | MRL/MpJ-Atp6v1b1vtx/Kjn | J:243701 | View | ||||
| enlarged vestibular aqueduct | Atp6v1b1vtx/Atp6v1b1vtx | MRL/MpJ-Atp6v1b1vtx/Kjn | J:243701 | View | ||||
| nonsyndromic deafness | Alg10bnse5/Alg10bnse5 | 129S6.B6-Alg10bnse5 | J:209766 | View | ||||
| nonsyndromic deafness | Klc2em1Jgao/Klc2em1Jgao | involves: C57BL/6J | J:345577 | View | ||||
| nonsyndromic deafness | m12Anu/m12Anu | Not Specified | J:207337 | View | ||||
| nonsyndromic deafness | Ppip5k2tm1a(EUCOMM)Wtsi/Ppip5k2tm1a(EUCOMM)Wtsi | involves: C57BL/6N | J:260825 | View | ||||
| nonsyndromic deafness | Wfs1tm1Koks/Wfs1tm1Koks | involves: 129S6/SvEvTac * C57BL/6J | J:342760 | View | ||||
| otitis media | Bpifa1m1Ingm/Bpifa1m1Ingm | involves: C3HeB/FeJ | J:221346 | View | ||||
| otitis media | Cby1tm1Ktkm/Cby1tm1Ktkm | B6.129-Cby1tm1Ktkm | J:147413 | View | ||||
| otitis media | Dnah5Tg1Htz/Dnah5Tg1Htz | involves: C57BL/6 * CBA/J | J:76189 | View | ||||
| otitis media | E2f4tm1Lees/E2f4tm1Lees | involves: 129S2/SvPas * C57BL/6 | J:64193 | View | ||||
| otitis media | Enpp1asj/Enpp1asj | C57BL/6J-Enpp1asj/GrsrJ | J:251638 | View | ||||
| otitis media | Eya4tm1Jse/Eya4tm1Jse | involves: 129S6/SvEvTac * CBA/J | J:131873 | View | ||||
| otitis media | Fbxo11Jf/Fbxo11+ | involves: BALB/cAnNCrl | J:250208 | View | ||||
| otitis media | Fbxo11Jf/Fbxo11+ | involves: BALB/cAnNCrl * C3H/HeN * C57BL/6J | J:114851 | View | ||||
| otitis media | Fbxo11Jf/Fbxo11Mutt | involves: BALB/c * C3H/HeN | J:114851 | View | ||||
| otitis media | Fgfr1Eask/Fgfr1+ | BALB/cByJ-Fgfr1Eask/GrsrJ | J:222308 | View | ||||
| otitis media | Fgfr1Hspy/Fgfr1+ | C3HeB/FeJ-Hspy | J:95895 | View | ||||
| otitis media | Iduatm1Efn/Iduatm1Efn | involves: C57BL/6 | J:123676 | View | ||||
| otitis media | LmnaDhe/Lmna+ | B6(D2)-LmnaDhe/TyGrsrJ | J:188496 | View | ||||
| otitis media | MecomJbo/Mecom+ | C3N.C-MecomJbo | J:113445 | View | ||||
| otitis media | MecomJbo/Mecom+ | involves: BALB/c | J:250208 | View | ||||
| otitis media | Naglutm1Efn/Naglutm1Efn | involves: 129S/SvEv * C57BL/6J | J:129390 | View | ||||
| otitis media |
Nf2tm1Gth/Nf2tm2Gth Tg(Mpz-cre)2Brn/0 |
involves: 129P2/OlaHsd * FVB/N | J:63264 | View | ||||
| otitis media |
Nf2tm2Gth/Nf2tm2Gth Tg(Mpz-cre)1Brn/0 |
involves: 129P2/OlaHsd * FVB/N | J:63264 | View | ||||
| otitis media |
Nf2tm2Gth/Nf2tm2Gth Tg(Mpz-cre)2Brn/0 |
involves: 129P2/OlaHsd * FVB/N | J:63264 | View | ||||
| otitis media | Nischedsn/Nischedsn | C3H.B6-Nischedsn | J:244907 | View | ||||
| otitis media | PhexHyp-Duk/Y | involves: BALB/cAnBomUrd | J:88352 | View | ||||
| otitis media | Rpl38Ts/Rpl38+ | TSJ/Le | J:168509 | View | ||||
| otitis media | Sall4tm1Brd/Sall4+ | involves: 129S7/SvEvBrd * C57BL/6J | J:117866 | View | ||||
| otitis media | Sh3pxd2bnee/Sh3pxd2bnee | B10.Cg-H2h4 Sh3pxd2bnee/GrsrJ | J:153369 | View | ||||
| otitis media | Tbx1tm1Bem/Tbx1+ | FVB.Cg-Tbx1tm1Bem | J:91664 | View | ||||
| otitis media | Tgif1tm1.1Caw/Tgif1tm1.1Caw | B6.129S-Tgif1tm1.1Caw | J:198238 | View | ||||
| otitis media | Trp73tm1Fmc/Trp73tm1Fmc | involves: 129S4/SvJae | J:60896 | View | ||||
| sensorineural hearing loss | Gas2tm1a(EUCOMM)Hmgu/Gas2tm1a(EUCOMM)Hmgu | C57BL/6N-Gas2tm1a(EUCOMM)Hmgu | J:306028 | View | ||||
| sensorineural hearing loss | Slc7a8tm1Geno/Slc7a8tm1Geno | involves: 129 * C57BL/6J | J:256844 | View | ||||
| sensorineural hearing loss | Slc7a14em1Jin/Slc7a14em1Jin | C57BL/6J-Slc7a14em1Jin | J:323825 | View | ||||
| sensorineural hearing loss | Slc7a14em1Jqu/Slc7a14em1Jqu | C57BL/6-Slc7a14em1Jqu | J:323825 | View | ||||
| sensorineural hearing loss | Slitrk6tm1.1Jaru/Slitrk6tm1.1Jaru | involves: 129P2/OlaHsd | J:169557 | View | ||||
| tympanosclerosis | Enpp1asj/Enpp1asj | C57BL/6J-Enpp1asj/GrsrJ | J:251638 | View | ||||
| vestibular disease | Kcna10tm1Lex/Kcna10tm1Lex | involves: 129S/SvEvBrd * C57BL/6 | J:210785 | View | ||||
| Transgenes and Other Mutations | autosomal recessive nonsyndromic deafness 38 | Phl1101/H/Phl1101/H | involves: 101/H * MAI/Pas | J:111682 | View | |||
| autosomal recessive nonsyndromic deafness 38 | Phl1101/H/Phl1101/H | involves: 101/H * MBT/Pas | J:111682 | View | ||||
| Additional Complex Models | otitis media | Ctnnb1tm1(Nfkbia)Rsu/Ctnnb1+ | involves: 129P2/OlaHsd * C57BL/6 | J:71744 | View | |||
| otitis media |
Ctnnb1tm2(Nfkbia)Rsu/Ctnnb1+ Tg(CMV-cre)1Cgn/0 |
involves: 129P2/OlaHsd * BALB/cJ * C57BL/6 | J:71744 | View | ||||
| otitis media |
Id1tm1Zhu/Id1+ Id3tm1Zhu/Id3tm1Zhu |
involves: 129S1/Sv * 129S4/SvJaeSor | J:311050 | View | ||||
| otitis media |
Id1tm1Zhu/Id1tm1Zhu Id3tm1Zhu/Id3+ |
involves: 129S1/Sv * 129S4/SvJaeSor | J:311050 | View | ||||
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No similarity to the expected human disease phenotype was found.
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| Disease Term | Allelic Composition | Genetic Background | Reference | Phenotypes | ||||
| NOT Models | autosomal dominant nonsyndromic deafness 2A | Gjb3tm1Kwi/Gjb3tm1Kwi | involves: 129S1/Sv * 129X1/SvJ * C57BL/6 | J:68014 | View | |||
| autosomal dominant nonsyndromic deafness 15 | Pou4f3tm1Rsd/Pou4f3+ | involves: 129S4/SvJae * C57BL/6 | J:57149 | View | ||||
| autosomal dominant nonsyndromic deafness 65 |
Cdh23Ahl+/Cdh23Ahl+ Tbc1d24em4Tbf/Tbc1d24em4Tbf |
B6.Cg-Cdh23Ahl+ Tbc1d24em4Tbf | J:299025 | View | ||||
| autosomal dominant nonsyndromic deafness 65 | Tbc1d24em4Tbf/Tbc1d24em4Tbf | C57BL/6J-Tbc1d24em4Tbf | J:299025 | View | ||||
| autosomal recessive nonsyndromic deafness 86 | Tbc1d24em3Tbf/Tbc1d24em3Tbf | C57BL/6J-Tbc1d24em3Tbf | J:299025 | View | ||||