B6.CBA-Del(14Gjb6-Cryl1)1Lmon/Cnbc
| Status | Available to order |
| EMMA ID | EM:15994 |
| Citation information | RRID:IMSR_EM:15994 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information. |
| International strain name | B6.CBA-Del(14Gjb6-Cryl1)1Lmon/Cnbc |
| Alternative name | B6/OlaHsd.CBA.Gjb6 |
| Strain type | Endonuclease-mediated |
| Allele/Transgene symbol | Del(14Gjb6-Cryl1)1Lmon |
| Gene/Transgene symbol | Del(14Gjb6-Cryl1)1Lmon |
Information from provider
| Provider | Lluis Montoliu |
| Provider affiliation | Centro Nacional de Biotecnología (CNB-CSIC) |
| Additional owner | Ignacio del Castillo. Servicio de Genética, Hospital Universitario Ramón y Cajal, IRYCIS, 28034 Madrid, Spain. |
| Genetic information | Murine model for a deletion starting at the Gjb6 gene and spanning 274 kb upstream, on mouse chromosome 14, removing the Gjb6 and Cryl1 genes. This deletion is homologous to the del(GJB6-D13S1830) deletion in humans, which is responsible for a phenotype of DFNB1 non-syndromic hearing loss (OMIM 220290, N. Engl. J. Med. 2002;346:243-9. doi: 10.1056/NEJMoa012052, PMID: 11807148). CRISPR technology details: Cas9 mRNA (Dharmacon, Lafayette, CO, USA) was used to form Cas9-RNP complexes. |
| Phenotypic information | Homozygous:Viable and fertile. Severe to profound hearing loss, with significantly increased auditory brainstem response (ABR) thresholds in response to click and tone-burst stimuli, compared to age-matched normal-hearing wild-type mice.Heterozygous:Viable, fertile and indistinguishable from their wild-type littermates. |
| Breeding history | CRISPR reagents were injected into mouse B6CBAF2 fertilized oocytes. Surviving embryos were transferred into two foster mothers, which eventually gave birth to 5 founder mice. One of the five founders, was able to transmit the deleted allele (Dfnb1em274) to the progeny. Heterozygous Dfnb1em274 mice were backcrossed to C57BL/6JOlaHsd for five consecutive generations. Upon reaching N5, heterozygous mice were subsequently intercrossed to generate Dfnb1em274 homozygotes. |
| References |
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| Homozygous fertile | yes |
| Homozygous viable | yes |
| Homozygous matings required | no |
| Immunocompromised | no |
Information from EMMA
| Archiving centre | CNB-CSIC, Centro Nacional de Biotecnologia, Madrid, Spain |
| Animals used for archiving | homozygous C57BL/6JOlaHsd males |
Literature references
- A murine model for the del(GJB6-D13S1830) deletion recapitulating the phenotype of human DFNB1 hearing impairment: generation and functional and histopathological study.;Domínguez-Ruiz María, Murillo-Cuesta Silvia, Contreras Julio, Cantero Marta, Garrido Gema, Martín-Bernardo Belén, Gómez-Rosas Elena, Fernández Almudena, Del Castillo Francisco J, Montoliu Lluís, Varela-Nieto Isabel, Del Castillo Ignacio, ;2024;BMC genomics;25;359; 38605287