C3HeB/FeJ-Six1Cwe/IegWtsiCnbc

Status

Available to order

EMMA IDEM:05016
International strain nameC3HeB/FeJ-Six1Cwe/IegWtsiCnbc
Alternative nameCatweasel
Strain typeInduced Mutant Strains : Chemically-induced
Allele/Transgene symbolSix1Cwe,
Gene/Transgene symbolSix1

Information from provider

ProviderKaren Steel
Provider affiliationWellcome Trust Sanger Institute
Additional ownerHelmholtz Zentrum Muenchen carried out the mutagenesis and discovered the head-bobing.
Genetic informationENU mutagenesis induced an A to G transition at position 411 resulting in an amino acid substitution of glycine for glutamic acid at position 135 (E135G). This mutation in the homeobox is predicted to cause destabilisation of DNA binding.
Phenotypic informationCwe/Cwe animals lack Preyer and righting reflexes, display severe headshaking and have severely truncated cochlea and semicircular canals. Cwe/Cwe animals had very few hair cells in the utricle, but their ampullae and cochlea were devoid of any hair cells. Heterozygotes show a mild head-bobbing behaviour.
Breeding historyOriginated from ENU mutagenesis programme in Munich on a C3HeB/FeJ background and maintained on the same background within a closed colony or backcrossed to C3HeB/FeJ ever since.
References
  • Catweasel mice: a novel role for Six1 in sensory patch development and a model for branchio-oto-renal syndrome.;Bosman Erika A, Quint Elizabeth, Fuchs Helmut, Hrabé de Angelis Martin, Steel Karen P, ;2009;Developmental biology;328;285-96; 19389353
Homozygous fertilenot known
Homozygous viableyes
Homozygous matings requiredno
Immunocompromisednot known

Information from EMMA

Archiving centreCNB-CSIC, Centro Nacional de Biotecnologia, Madrid, Spain

Disease and phenotype information

MGI allele-associated human disease models

Orphanet associated rare diseases, based on orthologous gene matching

MGI phenotypes (allele matching)
  • abnormal inner ear vestibule morphology / MGI
  • head shaking / MGI
  • increased cochlear inner hair cell number / MGI
  • abnormal otic vesicle development / MGI
  • abnormal cochlea morphology / MGI
  • decreased body weight / MGI
  • decreased body size / MGI
  • circling / MGI
  • limb grasping / MGI
  • impaired righting response / MGI
  • abnormal inner ear canal morphology / MGI
  • absent lateral semicircular canal / MGI
  • renal hypoplasia / MGI
  • abnormal crista ampullaris morphology / MGI
  • small vestibular saccule / MGI
  • absent incus / MGI
  • small utricle / MGI
  • decreased cochlear inner hair cell number / MGI
  • abnormal common crus morphology / MGI
  • inner ear hypoplasia / MGI
  • absent pinna reflex / MGI

Literature references

  • Catweasel mice: a novel role for Six1 in sensory patch development and a model for branchio-oto-renal syndrome.;Bosman Erika A, Quint Elizabeth, Fuchs Helmut, Hrabé de Angelis Martin, Steel Karen P, ;2009;Developmental biology;328;285-96; 19389353

Order

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*
  • Rederivation of mice from frozen stock, delivery time available upon request . €3880*

Due to the dynamic nature of our processes strain availability may change at short notice. The local repository manager will advise you in these circumstances.

* In addition users have to cover all the shipping costs (including the cost for returning dry-shippers, where applicable).

More details on pricing and delivery times

Practical information

Genotyping protocol

Example health report
(Current health report will be provided later)

Material Transfer Agreement (MTA)
Distribution of this strain is subject to a provider MTA. Both signing of the MTA and submission of the online EMMA Mutant Request Form are required before material can be shipped.

EMMA conditions
Legally binding conditions for the transfer

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