B6N.Cg-Gjb2tm1Ugds Tg(Sox10-cre)1Wdr/Cnrm
| Status | Available to order |
| EMMA ID | EM:11478 |
| International strain name | B6N.Cg-Gjb2tm1Ugds Tg(Sox10-cre)1Wdr/Cnrm |
| Alternative name | Cx26-Sox10Cre |
| Strain type | Transgenic Strains |
| Allele/Transgene symbol | Tg(Sox10-cre)1Wdr, |
| Gene/Transgene symbol | Tg(Sox10-cre)1Wdr |
Information from provider
| Provider | Fabio Mammano |
| Provider affiliation | IBCN, CNR |
| Genetic information | These mice, with targeted ablation of connexin 26 (Gjb2) in non-sensory cells of the inner ear were generated by crossing Cx26 loxP/loxP mice (MGI:2183509) with Sox10-Cre mice (MGI:3586900). |
| Phenotypic information | Homozygous:These mice present with elevated hearing thresholds (>90 dB SPL) at all tested frequencies.Heterozygous:No obvious developmental or behavioural abnormality. Normal hearing at weaning. |
| Breeding history | N=5 backcrosses to C57BL/6N. |
| References |
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| Homozygous fertile | yes |
| Homozygous viable | yes |
| Homozygous matings required | no |
| Immunocompromised | no |
Information from EMMA
| Archiving centre | CNR, Consiglio Nazionale delle Ricerche, Monterotondo, Italy |
Disease and phenotype information
MGI allele-associated human disease models
Orphanet associated rare diseases, based on orthologous gene matching
- Porokeratotic eccrine ostial and dermal duct nevus / Orphanet_166286
- Palmoplantar keratoderma-deafness syndrome / Orphanet_2202
- Knuckle pads-leukonychia-sensorineural deafness-palmoplantar hyperkeratosis syndrome / Orphanet_2698
- Autosomal recessive non-syndromic sensorineural deafness type DFNB / Orphanet_90636
- Keratoderma hereditarium mutilans / Orphanet_494
- KID syndrome / Orphanet_477
- Autosomal dominant non-syndromic sensorineural deafness type DFNA / Orphanet_90635
Literature references
- ATP release through connexin hemichannels and gap junction transfer of second messengers propagate Ca2+ signals across the inner ear.;Anselmi Fabio, Hernandez Victor H, Crispino Giulia, Seydel Anke, Ortolano Saida, Roper Stephen D, Kessaris Nicoletta, Richardson William, Rickheit Gesa, Filippov Mikhail A, Monyer Hannah, Mammano Fabio, ;2008;Proceedings of the National Academy of Sciences of the United States of America;105;18770-5; 19047635
- BAAV mediated GJB2 gene transfer restores gap junction coupling in cochlear organotypic cultures from deaf Cx26Sox10Cre mice.;Crispino Giulia, Di Pasquale Giovanni, Scimemi Pietro, Rodriguez Laura, Galindo Ramirez Fabian, De Siati Romolo Daniele, Santarelli Rosa Maria, Arslan Edoardo, Bortolozzi Mario, Chiorini John A, Mammano Fabio, ;2011;PloS one;6;e23279; 21876744
- Connexin-Mediated Signaling in Nonsensory Cells Is Crucial for the Development of Sensory Inner Hair Cells in the Mouse Cochlea.;Johnson Stuart L, Ceriani Federico, Houston Oliver, Polishchuk Roman, Polishchuk Elena, Crispino Giulia, Zorzi Veronica, Mammano Fabio, Marcotti Walter, ;2017;The Journal of neuroscience : the official journal of the Society for Neuroscience;37;258-268; 28077706
- Inner Ear Connexin Channels: Roles in Development and Maintenance of Cochlear Function.;Mammano Fabio, ;2019;Cold Spring Harbor perspectives in medicine;9;301-317; 30181354
- Cx26 partial loss causes accelerated presbycusis by redox imbalance and dysregulation of Nfr2 pathway.;Fetoni Anna Rita, Zorzi Veronica, Paciello Fabiola, Ziraldo Gaia, Peres Chiara, Raspa Marcello, Scavizzi Ferdinando, Salvatore Anna Maria, Crispino Giulia, Tognola Gabriella, Gentile Giulia, Spampinato Antonio Gianmaria, Cuccaro Denis, Guarnaccia Maria, Morello Giovanna, Van Camp Guy, Fransen Erik, Brumat Marco, Girotto Giorgia, Paludetti Gaetano, Gasparini Paolo, Cavallaro Sebastiano, Mammano Fabio, ;2018;Redox biology;19;117-126; 30199819
- Ca2+ signaling, apoptosis and autophagy in the developing cochlea: Milestones to hearing acquisition.;Mammano Fabio, Bortolozzi Mario, ;2018;Cell calcium;70;; 28578918