IMPC phenotypes (gene matching)
129S2(Cg)-Dcxtm1.2Ffr/Orl
| Status | Available to order |
| EMMA ID | EM:05808 |
| International strain name | 129S2(Cg)-Dcxtm1.2Ffr/Orl |
| Alternative name | Dcx knockout Sv129Pas |
| Strain type | Targeted Mutant Strains : Knock-out |
| Allele/Transgene symbol | Dcxtm1.2Ffr |
| Gene/Transgene symbol | Dcx |
Information from provider
| Provider | Fiona Francis |
| Provider affiliation | Inserm UMRS 839 |
| Genetic information | To generate Dcx knock-out 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 exon 3. 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 which will further allow the inactivation of Dcx in a spatially and temporally controlled manner. Mice are maintained on pure C57BL/6N and 129/SvPas backgrounds. We would like to cryopreserve knock-out mice on the 129/SvPas background. The Dcx gene is on the X chromosome and hence knock-out males are hemizygotes. Crosses with wild-type 129/SvPas females will produce heterozygous females and wild-type males. |
| Phenotypic information | These constitutive knockout mice have hippocampus lamination defects and are a model of hippocampus dysplasia associated with epilepsy. |
| Breeding history | Backcrossing has been performed for at least 10 generations. For cryoconservation we would use hemizygous males crossed with wild-type females. |
| References |
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| Homozygous fertile | yes |
| Homozygous viable | yes |
| Homozygous matings required | no |
| Immunocompromised | no |
Information from EMMA
| Archiving centre | Institut de Transgenose, INTRAGENE, Orléans, France |
| Animals used for archiving | heterozygous 129S2/SvPas |
| Breeding at archiving centre | Hemizygous males with wild-type females |
| Stage of embryos | 2-cell |
Disease and phenotype information
MGI allele-associated human disease models
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