IMPC phenotypes (gene matching)
B6N.129S2(Cg)-Dcxtm1.1Ffr/Orl
| Status | Available to order |
| EMMA ID | EM:05807 |
| Citation information | RRID:IMSR_EM:05807 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 | B6N.129S2(Cg)-Dcxtm1.1Ffr/Orl |
| Alternative name | Dcx flox C57BL/6N |
| Strain type | Targeted Mutant Strains : Conditional mutation |
| Allele/Transgene symbol | Dcxtm1.1Ffr |
| Gene/Transgene symbol | Dcx |
Information from provider
| Provider | Fiona Francis |
| Provider affiliation | Inserm UMRS 839 |
| Genetic information | To generate floxed Dcx mice (Kappeler et al, 2006) a loxP site containing a BamHI restriction site was inserted in the BsaI site upstream of Dcx exon 3. A floxed PGK-neo selection gene was inserted into the XbaI site downstream of this exon. Mice carrying a floxed Dcx allele, with loxP sites flanking exon 3 and a floxed selection gene, were crossed with cre transgenic mice expressing the cre recombinase early in development (Leneuve et al, 2003). This cross generated (1) knock-out mice (having deleted Dcx exon 3 and the selection gene) and (2) mice deleted for the selection gene but still carrying the floxed exon 3 (Dcx flox mice) which will further allow the inactivation of Dcx in a spatially and temporally controlled manner. Mice are maintained on a pure C57BL/6N background. The Dcx gene is on the X chromosome and hence floxed male mice are hemizygotes. Crosses with wild-type C57BL/6N females will produce heterozygous female and wild-type male embryos. Dcx flox homozygotes are also viable and fertile. |
| Phenotypic information | The floxed Dcx allele mice have no detectable phenotype. Constitutive knock-out mice have hippocampus lamination defects and are a model of hippocampus dysplasia associated with epilepsy. |
| Breeding history | Initially backcrossing was performed for at least 10 generations. Then heterozygous females were crossed with hemizygous males to generate homozygotes. For cryoconservation we would use male hemizygous males crossed with wild-type females. |
| References |
|
| Homozygous fertile | yes |
| Homozygous viable | yes |
| Homozygous matings required | no |
| Immunocompromised | no |
Information from EMMA
| Archiving centre | CNRS-TAAM – Typing and Archiving of Animal Models, Orléans, France |
| Animals used for archiving | heterozygous C57BL/6N males |
Disease and phenotype information
Orphanet associated rare diseases, based on orthologous gene matching
- Lissencephaly type 1 due to doublecortin gene mutation / Orphanet_2148
- Subcortical band heterotopia / Orphanet_99796
Literature references
- Branching and nucleokinesis defects in migrating interneurons derived from doublecortin knockout mice.;Kappeler Caroline, Saillour Yoann, Baudoin Jean-Pierre, Tuy Françoise Phan Dinh, Alvarez Chantal, Houbron Christophe, Gaspar Patricia, Hamard Ghislaine, Chelly Jamel, Métin Christine, Francis Fiona, ;2006;Human molecular genetics;15;1387-400; 16571605
- Magnetic resonance imaging and histological studies of corpus callosal and hippocampal abnormalities linked to doublecortin deficiency.;Kappeler Caroline, Dhenain Marc, Phan Dinh Tuy Françoise, Saillour Yoann, Marty Serge, Fallet-Bianco Catherine, Souville Isabelle, Souil Evelyne, Pinard Jean-Marc, Meyer Gundela, Encha-Razavi Ferechté, Volk Andreas, Beldjord Cherif, Chelly Jamel, Francis Fiona, ;2007;The Journal of comparative neurology;500;239-54; 17111359
- Epilepsy in Dcx knockout mice associated with discrete lamination defects and enhanced excitability in the hippocampus.;Nosten-Bertrand Marika, Kappeler Caroline, Dinocourt Céline, Denis Cécile, Germain Johanne, Phan Dinh Tuy Françoise, Verstraeten Soraya, Alvarez Chantal, Métin Christine, Chelly Jamel, Giros Bruno, Miles Richard, Depaulis Antoine, Francis Fiona, ;2008;PloS one;3;e2473; 18575605
- Cellular anatomy, physiology and epileptiform activity in the CA3 region of Dcx knockout mice: a neuronal lamination defect and its consequences.;Bazelot Michael, Simonnet Jean, Dinocourt Céline, Bruel-Jungerman Elodie, Miles Richard, Fricker Desdemona, Francis Fiona, ;2012;The European journal of neuroscience;35;244-56; 22250815