D2.Cg-Cdkl5tm1.2Cogr/Cnrm

Status

Under development - register interest

EMMA IDEM:09061
International strain nameD2.Cg-Cdkl5tm1.2Cogr/Cnrm
Alternative nameCDKL5
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolCdkl5tm1.2Cogr,
Gene/Transgene symbolCdkl5

Information from provider

ProviderCornelius Gross
Provider affiliationDevelopmental programming of behaviour, EMBL
Genetic informationCdkl5 exon 4 was targeted by homologous recombination. Exon 4 was flanked by two loxP sites followed by neomycin cassette flanked by FRT sites used for removal of the cassette. Hprt-cre mice were used to remove exon 4 to generate Cdkl5 knock-out mice.
Phenotypic informationHomozygous:
Heterozygous and homozygous knock-out mice displayed limb clasping, and motor and respiratory disturbances at approximately six weeks of age. Dendritic arborization was significantly reduced in cortical neurons from knock-out mice and evidence emerged for altered signaling in key intracellular pathways.

Heterozygous:
Heterozygous and homozygous knock-out mice displayed limb clasping, and motor and respiratory disturbances at approximately six weeks of age. Dendritic arborization was significantly reduced in cortical neurons from knock-out mice and evidence emerged for altered signaling in key intracellular pathways.
Breeding historyMutant mice were crossed with C57BL/6J flp-deleter mice (Farley et al., 2000) made congenic in B6N genetic background (N10). Then mice were crossed with C57BL/6J cre-deleter mice (Tang et al., 2002) made congenic in B6N genetic background (N10). After that mice were bred for 7 generations in a C57BL/6N genetic background. The first 2 matings (flp-deleter and cre-deleter strains) were not considered in the calculation of the generations (Amendola et al., PLoS One 2014). Finally the mice were crossed with DBA/2NCrl for 10 generations.
References
  • Mapping pathological phenotypes in a mouse model of CDKL5 disorder.;Amendola Elena, Zhan Yang, Mattucci Camilla, Castroflorio Enrico, Calcagno Eleonora, Fuchs Claudia, Lonetti Giuseppina, Silingardi Davide, Vyssotski Alexei L, Farley Dominika, Ciani Elisabetta, Pizzorusso Tommaso, Giustetto Maurizio, Gross Cornelius T, ;2014;PloS one;9;e91613; 24838000
Homozygous fertilenot known
Homozygous viablenot known
Homozygous matings requiredno
Immunocompromisedno

Information from EMMA

Archiving centreCNR, 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

MGI phenotypes (allele matching)
  • hypoactivity / MGI
  • limb grasping / MGI
  • abnormal visual evoked potential / MGI
  • seizures / MGI
  • abnormal seizure response to inducing agent / MGI

Literature references

  • Mapping pathological phenotypes in a mouse model of CDKL5 disorder.;Amendola Elena, Zhan Yang, Mattucci Camilla, Castroflorio Enrico, Calcagno Eleonora, Fuchs Claudia, Lonetti Giuseppina, Silingardi Davide, Vyssotski Alexei L, Farley Dominika, Ciani Elisabetta, Pizzorusso Tommaso, Giustetto Maurizio, Gross Cornelius T, ;2014;PloS one;9;e91613; 24838000

Register interest

Availabilities

Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

  • Frozen embryos. Delivered in 4 weeks (after paperwork in place). €1740*
  • Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740*
  • Rederivation of mice from frozen stock, delivery time available upon request . €3880*

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Practical information

Example health report
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