Excel File Text File All mouse models of ciliopathy with phenotypic similarity to the human disease
        Disease Term Allelic Composition Genetic Background Reference Phenotypes
      Joubert syndrome 3 Ahi1tm1Jgg/Ahi1tm1Jgg involves: 129S1/Sv * 129X1/SvJ J:172420 View
Joubert syndrome 5 Cep290Gt(CC0582)Wtsi/Cep290Gt(CC0582)Wtsi 129P2/OlaHsd-Cep290Gt(CC0582)Wtsi J:212182, J:226246 View
Joubert syndrome 5 Cep290tm1.1Jgg/Cep290tm1.1Jgg involves: 129 J:172420 View
Joubert syndrome 6 Tmem67tm1Dgen/Tmem67tm1Dgen B6.129P2-Tmem67tm1Dgen J:193837 View
Joubert syndrome 7 Rpgrip1ltm1Urt/Rpgrip1ltm1Urt involves: 129S1/Sv * 129X1/SvJ J:123018 View
Joubert syndrome 17 Cplane1b2b012Clo/Cplane1b2b012Clo C57BL/6J-Cplane1b2b012Clo J:223340 View
Joubert syndrome 26 KatnipGt(RRG309)Byg/KatnipGt(RRG309)Byg involves: 129P2/OlaHsd * C57BL/6 J:239987 View
Kartagener syndrome Dnah5b2b002Clo/Dnah5b2b002Clo C57BL/6J-Dnah5b2b002Clo J:175213 View
Kartagener syndrome Dnah5b2b016Clo/Dnah5b2b016Clo C57BL/6J-Dnah5b2b016Clo J:175213 View
Kartagener syndrome Dnah5b2b601Clo/Dnah5b2b601Clo C57BL/6J-Dnah5b2b601Clo J:175213 View
Kartagener syndrome Dnah5b2b1003Clo/Dnah5b2b1003Clo C57BL/6J-Dnah5b2b1003Clo J:175213 View
Kartagener syndrome Dnah5b2b1134Clo/Dnah5b2b1134Clo C57BL/6J-Dnah5b2b1134Clo J:175213 View
Kartagener syndrome Dnah5b2b1154Clo/Dnah5b2b1154Clo C57BL/6J-Dnah5b2b1154Clo J:175213 View
Kartagener syndrome Dnah5b2b1537Clo/Dnah5b2b1537Clo C57BL/6J-Dnah5b2b1537Clo J:175213 View
Kartagener syndrome Dnah5b2b1565Clo/Dnah5b2b1565Clo C57BL/6J-Dnah5b2b1565Clo J:175213 View
Kartagener syndrome Dnah5b2b2395Clo/Dnah5b2b2395Clo C57BL/6J-Dnah5b2b2395Clo J:175213 View
Kartagener syndrome Dnah5b2b2570Clo/Dnah5b2b2570Clo C57BL/6J-Dnah5b2b2570Clo J:175213 View
Kartagener syndrome Dnah5b2b2925Clo/Dnah5b2b2925Clo C57BL/6J-Dnah5b2b2925Clo J:175213 View
Kartagener syndrome Dnah5b2b3491Clo/Dnah5b2b3491Clo C57BL/6J-Dnah5b2b3491Clo J:175213 View
Kartagener syndrome Dnah11b2b1727Clo/Dnah11b2b1727Clo C57BL/6J-Dnah11b2b1727Clo J:175213 View
Kartagener syndrome Dnah11b2b2349Clo/Dnah11b2b2349Clo C57BL/6J-Dnah11b2b2349Clo J:175213 View
Kartagener syndrome Dnai1b2b1526Clo/Dnai1b2b1526Clo C57BL/6J-Dnai1b2b1526Clo J:175213 View
Meckel syndrome B9d1tm1a(EUCOMM)Wtsi/B9d1tm1a(EUCOMM)Wtsi involves: C57BL/6N * CD-1 J:178421 View
primary ciliary dyskinesia Daw1b2b1116Clo/Daw1b2b1116Clo C57BL/6J-Daw1b2b1116Clo J:175213 View
primary ciliary dyskinesia Daw1b2b1584Clo/Daw1b2b1584Clo C57BL/6J-Daw1b2b1584Clo J:175213 View
primary ciliary dyskinesia 1 Dnai1b2b284Clo/Dnai1b2b284Clo C57BL/6J-Dnai1b2b284Clo J:175213 View
primary ciliary dyskinesia 1 Dnai1b2b1526Clo/Dnai1b2b1526Clo C57BL/6J-Dnai1b2b1526Clo J:175213 View
primary ciliary dyskinesia 1 Dnai1tm1.1Leo/Dnai1tm1.1Leo
Gt(ROSA)26Sortm1(cre/ERT)Nat/Gt(ROSA)26Sor+		 involves: 129 * C57BL/6 J:155730 View
primary ciliary dyskinesia 2 Dnaaf3b2b1739Clo/Dnaaf3b2b1739Clo C57BL/6J-Dnaaf3b2b1739Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b002Clo/Dnah5b2b002Clo C57BL/6J-Dnah5b2b002Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b016Clo/Dnah5b2b016Clo C57BL/6J-Dnah5b2b016Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b601Clo/Dnah5b2b601Clo C57BL/6J-Dnah5b2b601Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b1003Clo/Dnah5b2b1003Clo C57BL/6J-Dnah5b2b1003Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b1134Clo/Dnah5b2b1134Clo C57BL/6J-Dnah5b2b1134Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b1154Clo/Dnah5b2b1154Clo C57BL/6J-Dnah5b2b1154Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b1537Clo/Dnah5b2b1537Clo C57BL/6J-Dnah5b2b1537Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b1565Clo/Dnah5b2b1565Clo C57BL/6J-Dnah5b2b1565Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b2395Clo/Dnah5b2b2395Clo C57BL/6J-Dnah5b2b2395Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b2570Clo/Dnah5b2b2570Clo C57BL/6J-Dnah5b2b2570Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b2925Clo/Dnah5b2b2925Clo C57BL/6J-Dnah5b2b2925Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5b2b3491Clo/Dnah5b2b3491Clo C57BL/6J-Dnah5b2b3491Clo J:175213 View
primary ciliary dyskinesia 3 Dnah5hlb612/Dnah5hlb612 involves: C57BL/6J J:130755 View
primary ciliary dyskinesia 3 Dnah5Tg1Htz/Dnah5Tg1Htz involves: C57BL/6 * CBA/J J:76189 View
primary ciliary dyskinesia 7 Dnah11b2b598Clo/Dnah11b2b598Clo C57BL/6J-Dnah11b2b598Clo J:175213 View
primary ciliary dyskinesia 7 Dnah11b2b1203Clo/Dnah11b2b1203Clo C57BL/6J-Dnah11b2b1203Clo J:175213 View
primary ciliary dyskinesia 7 Dnah11b2b1279Clo/Dnah11b2b1279Clo C57BL/6J-Dnah11b2b1279Clo J:175213 View
primary ciliary dyskinesia 7 Dnah11b2b1289Clo/Dnah11b2b1289Clo C57BL/6J-Dnah11b2b1289Clo J:175213 View
primary ciliary dyskinesia 7 Dnah11b2b1727Clo/Dnah11b2b1727Clo C57BL/6J-Dnah11b2b1727Clo J:175213 View
primary ciliary dyskinesia 7 Dnah11b2b1775Clo/Dnah11b2b1775Clo C57BL/6J-Dnah11b2b1775Clo J:175213 View
primary ciliary dyskinesia 7 Dnah11b2b2349Clo/Dnah11b2b2349Clo C57BL/6J-Dnah11b2b2349Clo J:175213 View
primary ciliary dyskinesia 7 Dnah11iv/Dnah11iv involves: C3H J:212 View
primary ciliary dyskinesia 10 Dnaaf2tm1.1(KOMP)Vlcg/Dnaaf2tm1.1(KOMP)Vlcg B6N(Cg)-Dnaaf2tm1.1(KOMP)Vlcg/J J:278736 View
primary ciliary dyskinesia 10 Dnaaf2tm1.1Mkis/Dnaaf2tm1.1Mkis involves: C57BL/6 * C57BL/6NCrlj * CBA/JNCrlj J:201474 View
primary ciliary dyskinesia 14 Ccdc39b2b1304Clo/Ccdc39b2b1304Clo C57BL/6J-Ccdc39b2b1304Clo J:175213 View
primary ciliary dyskinesia 14 Ccdc39b2b1735Clo/Ccdc39b2b1735Clo C57BL/6J-Ccdc39b2b1735Clo J:175213 View
primary ciliary dyskinesia 14 Ccdc39b2b2025.1Clo/Ccdc39b2b2025.1Clo C57BL/6J-Ccdc39b2b2025.1Clo J:175213 View
primary ciliary dyskinesia 19 Dnaaf11tm1.2Hmd/Dnaaf11tm1.2Hmd involves: 129S6/SvEvTac * C57BL/6NCrl J:258369 View
primary ciliary dyskinesia 21 Drc1b2b1654Clo/Drc1b2b1654Clo C57BL/6J-Drc1b2b1654Clo J:175213 View
primary ciliary dyskinesia 21 Drc1b2b2237Clo/Drc1b2b2237Clo C57BL/6J-Drc1b2b2237Clo J:175213 View
primary ciliary dyskinesia 22 Zmynd10tm1(KOMP)Wtsi/Zmynd10tm1(KOMP)Wtsi involves: C57BL/6J * C57BL/6N J:261915 View
primary ciliary dyskinesia 23 Odad2b2b227.1Clo/Odad2b2b227.1Clo C57BL/6J-Odad2b2b227.1Clo J:175213 View
primary ciliary dyskinesia 23 Odad2b2b643Clo/Odad2b2b643Clo C57BL/6J-Odad2b2b643Clo J:175213, J:206684 View
primary ciliary dyskinesia 25 Dnaaf4tm1.2Jjlo/Dnaaf4tm1.2Jjlo involves: 129S1/Sv * 129S4/SvJaeSor * 129S6/SvEvTac * C57BL/6J J:205312 View
primary ciliary dyskinesia 30 Odad3b2b1885Clo/Odad3b2b1885Clo C57BL/6J-Odad3b2b1885Clo J:175213 View
primary ciliary dyskinesia 30 Odad3tm1b(EUCOMM)Hmgu/Odad3tm1b(EUCOMM)Hmgu C57BL/6N-Odad3tm1b(EUCOMM)Hmgu/Cnrm J:278798 View
primary ciliary dyskinesia 33 Gas8Gt(CH0760)Wtsi/Gas8em1Bky involves: 129P2/OlaHsd * C57BL/6 J:234173 View
primary ciliary dyskinesia 33 Gas8Gt(CH0760)Wtsi/Gas8Gt(CH0760)Wtsi involves: 129P2/OlaHsd * C57BL/6 J:234173 View
primary ciliary dyskinesia 35 Odad4em#Omra/Odad4em#Omra involves: C57BL/6 J:244361 View
      ciliopathy Kif7b2b2254Clo/Kif7b2b2254Clo C57BL/6J-Kif7b2b2254Clo J:175213 View
ciliopathy Sclt1Tg(CAG-sb10)1Dla/Sclt1Tg(CAG-sb10)1Dla FVB/N-Sclt1Tg(CAG-sb10)1Dla J:243427 View
ciliopathy Spry2tm1.1Mrt/Spry2+
Spry4tm1.2Mrt/Spry4tm1.2Mrt		 involves: 129P2/OlaHsd J:315670 View
ciliopathy Spry2tm1.1Mrt/Spry2tm1.1Mrt involves: 129P2/OlaHsd J:315670 View
ciliopathy Spry2tm1.1Mrt/Spry2tm1.1Mrt
Spry4tm1.2Mrt/Spry4+		 involves: 129P2/OlaHsd J:315670 View
ciliopathy Wdr11Gt(Ayu21-KBW205)Imeg/Wdr11Gt(Ayu21-KBW205)Imeg B6.Cg-Wdr11Gt(Ayu21-KBW205)Imeg J:257035 View
ciliopathy Wdr19twto/Wdr19twto involves: FVB/NJ J:181888 View
Joubert syndrome Zic1tm1Jaru/Zic1+ B6.129S4-Zic1tm1Jaru J:72342 View
Kartagener syndrome Ap1b1b2b1660Clo/Ap1b1b2b1660Clo C57BL/6J-Ap1b1b2b1660Clo J:175213 View
Kartagener syndrome b2b1200Clo/b2b1200Clo C57BL/6J-b2b1200Clo J:175213 View
Kartagener syndrome b2b1519Clo/b2b1519Clo C57BL/6J-b2b1519Clo J:175213 View
Kartagener syndrome b2b1700Clo/b2b1700Clo C57BL/6J-b2b1700Clo J:175213 View
Kartagener syndrome b2b2350Clo/b2b2350Clo C57BL/6J-b2b2350Clo J:175213 View
Kartagener syndrome Ccdc39b2b1304Clo/Ccdc39b2b1304Clo C57BL/6J-Ccdc39b2b1304Clo J:175213 View
Kartagener syndrome Ccdc39b2b1735Clo/Ccdc39b2b1735Clo C57BL/6J-Ccdc39b2b1735Clo J:175213 View
Kartagener syndrome Daw1b2b1116Clo/Daw1b2b1116Clo C57BL/6J-Daw1b2b1116Clo J:175213 View
Kartagener syndrome Daw1b2b1584Clo/Daw1b2b1584Clo C57BL/6J-Daw1b2b1584Clo J:175213 View
Kartagener syndrome Dnaaf3b2b1739Clo/Dnaaf3b2b1739Clo C57BL/6J-Dnaaf3b2b1739Clo J:175213 View
Kartagener syndrome Dnai2b2b3405Clo/Dnai2b2b3405Clo C57BL/6J-Dnai2b2b3405Clo J:175213 View
Kartagener syndrome Drc1b2b1654Clo/Drc1b2b1654Clo C57BL/6J-Drc1b2b1654Clo J:175213 View
Kartagener syndrome Drc1b2b2237Clo/Drc1b2b2237Clo C57BL/6J-Drc1b2b2237Clo J:175213 View
Kartagener syndrome Foxj1tm1Bph/Foxj1tm1Bph involves: 129S1/Sv * 129X1/SvJ * Black Swiss J:50025 View
Kartagener syndrome Foxj1tm1Slb/Foxj1tm1Slb involves: 129X1/SvJ J:73616 View
Kartagener syndrome Gm572b2b1167Clo/Gm572b2b1167Clo C57BL/6J-Gm572b2b1167Clo J:175213 View
Kartagener syndrome Odad2b2b643Clo/Odad2b2b643Clo C57BL/6J-Odad2b2b643Clo J:175213 View
Kartagener syndrome Odad3b2b1885Clo/Odad3b2b1885Clo C57BL/6J-Odad3b2b1885Clo J:175213 View
Kartagener syndrome Slit2b2b1200.1Clo/Slit2b2b1200.1Clo C57BL/6J-Slit2b2b1200.1Clo J:175213 View
Meckel syndrome bpck/bpck B6C3Fe a/a-bpck/J J:144929 View
Meckel syndrome Cc2d2atm1Asw/Cc2d2atm1Asw Not Specified J:226310 View
Meckel syndrome Cep55em1.1Hyli/Cep55em1.1Hyli involves: C57BL/6 J:313163 View
Meckel syndrome Mks1hlb614/Mks1hlb614 involves: C3H * C57BL/6 J:167534 View
Meckel syndrome Mks1krc/Mks1krc involves: C3HeB/FeJ * C57BL/6 * CD-1 J:154075 View
Meckel syndrome Mks1tm1a(EUCOMM)Wtsi/Mks1tm1a(EUCOMM)Wtsi involves: C57BL/6 * C57BL/6N J:196290 View
Meckel syndrome Rpgrip1ltm1Urt/Rpgrip1ltm1Urt involves: 129S1/Sv * 129X1/SvJ J:123018 View
Meckel syndrome Tbc1d32b2b2284Clo/Tbc1d32b2b2284Clo C57BL/6J-Tbc1d32b2b2284Clo J:175213 View
Meckel syndrome Tmem67tm1Dgen/Tmem67tm1Dgen B6.129P2-Tmem67tm1Dgen J:193837 View
primary ciliary dyskinesia Ap1b1b2b1660Clo/Ap1b1b2b1660Clo C57BL/6J-Ap1b1b2b1660Clo J:175213 View
primary ciliary dyskinesia b2b227Clo/b2b227Clo C57BL/6J-b2b227Clo J:175213 View
primary ciliary dyskinesia b2b1146Clo/b2b1146Clo C57BL/6J-b2b1146Clo J:175213 View
primary ciliary dyskinesia b2b1200Clo/b2b1200Clo C57BL/6J-b2b1200Clo J:175213 View
primary ciliary dyskinesia b2b1700Clo/b2b1700Clo C57BL/6J-b2b1700Clo J:175213 View
primary ciliary dyskinesia b2b1929Clo/b2b1929Clo C57BL/6J-b2b1929Clo J:175213 View
primary ciliary dyskinesia b2b2059Clo/b2b2059Clo C57BL/6J-b2b2059Clo J:175213 View
primary ciliary dyskinesia b2b2350Clo/b2b2350Clo C57BL/6J-b2b2350Clo J:175213 View
primary ciliary dyskinesia Cby1tm1Ktkm/Cby1tm1Ktkm B6.129-Cby1tm1Ktkm J:147413, J:166685 View
primary ciliary dyskinesia Del(19Poll-Dcpd)1Nmt/Del(19Poll-Dcpd)1Nmt involves: 129P2/OlaHsd J:75779 View
primary ciliary dyskinesia Dnaaf5em1Slb/Dnaaf5em1Slb C57BL/6-Dnaaf5em1Slb J:342770 View
primary ciliary dyskinesia Dnah5b2b2451Clo/Dnah5b2b2451Clo C57BL/6J-Dnah5b2b2451Clo J:175213 View
primary ciliary dyskinesia Dnai2b2b3405Clo/Dnai2b2b3405Clo C57BL/6J-Dnai2b2b3405Clo J:175213 View
primary ciliary dyskinesia Dpcd/PollGt(OST280355)Lex/Dpcd/PollGt(OST280355)Lex involves: 129S5/SvEvBrd * C57BL/6Brd J:171184 View
primary ciliary dyskinesia Foxj1tm1Bph/Foxj1tm1Bph involves: 129S1/Sv * 129X1/SvJ * Black Swiss J:50025 View
primary ciliary dyskinesia Foxj1tm1Slb/Foxj1tm1Slb involves: 129X1/SvJ J:73616 View
primary ciliary dyskinesia Gas2l2tm1c(KOMP)Wtsi/Gas2l2tm1c(KOMP)Wtsi
Foxj1tm1.1(cre/ERT2/GFP)Htg/Foxj1+		 involves: 129P2/OlaHsd * 129S4/SvJaeSor * C57BL/6N J:277289 View
primary ciliary dyskinesia Gm572b2b1167Clo/Gm572b2b1167Clo C57BL/6J-Gm572b2b1167Clo J:175213 View
primary ciliary dyskinesia Pcsk5b2b1549Clo/Pcsk5b2b1549Clo C57BL/6J-Pcsk5b2b1549Clo J:175213 View
primary ciliary dyskinesia Slit2b2b1200.1Clo/Slit2b2b1200.1Clo C57BL/6J-Slit2b2b1200.1Clo J:175213 View
primary ciliary dyskinesia Spef2bgh/Spef2bgh involves: 129S6/SvEvTac * C57BL/6J * C57BL/10J J:176695 View
primary ciliary dyskinesia Spef2bgh/Spef2bgh involves: C57BL/6J * C57BL/10J J:176695 View
primary ciliary dyskinesia Ttll1tm1Seto/Ttll1tm1Seto involves: 129S/SvEv * C57BL/6 J:161297 View
primary ciliary dyskinesia 14 b2b2025Clo/b2b2025Clo C57BL/6J-b2b2025Clo J:175213 View
Transgenes and
Other Mutations		     primary ciliary dyskinesia Del(1)1Brk/Del(1)1Brk involves: 129S6/SvEvTac * C57BL/6J * CBA/J J:130396 View
Additional
Complex
Models		     primary ciliary dyskinesia Ak7Tg(tetO-Hmox1)67Sami/Ak7Tg(tetO-Hmox1)67Sami FVB/N-Ak7Tg(tetO-Hmox1)67Sami J:186062 View

       
No similarity to the expected human disease phenotype was found.
        Disease Term Allelic Composition Genetic Background Reference Phenotypes
NOT Models      Stromme syndrome Cenpfem3Bko/Cenpfem3Bko involves: C57BL/6 * C57BL/6NTac * DBA/2 J:273098 View