B6.129S2-Otogtm1Prs/Orl

Status

Available to order

EMMA IDEM:01820
International strain nameB6.129S2-Otogtm1Prs/Orl
Alternative nameOtogC57BL/6J
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolOtogtm1Prs
Gene/Transgene symbolOtog

Information from provider

ProviderSIMMLER MARIE-CHRISTINE
Provider affiliationInstitut Jacques-Monod, CNRS, Equipe labellisée INSERM U950
Genetic informationTo inactivate the Otog gene the first three exons were removed and replaced with the lacZ gene, fused in-frame with the Otog translation initiation codon.
Phenotypic informationIn Otog -/- mice, both vestibular and auditory functions were impaired.
References
  • Twister mutant mice are defective for otogelin, a component specific to inner ear acellular membranes;Simmler MC, Zwaenepoel I, Verpy E, Guillaud L, Elbaz C, Petit C, Panthier JJ;2000;Mammalian genome : official journal of the International Mammalian Genome Society;11;960-6; 11063250
  • Targeted disruption of otog results in deafness and severe imbalance.;Simmler M C, Cohen-Salmon M, El-Amraoui A, Guillaud L, Benichou J C, Petit C, Panthier J J, ;2000;Nature genetics;24;139-43; 10655058

Information from EMMA

Archiving centreInstitut de Transgenose, INTRAGENE, Orléans, France

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

    • Autosomal recessive non-syndromic sensorineural deafness type DFNB / Orphanet_90636
IMPC phenotypes (gene matching)
  • abnormal auditory brainstem response / IMPC
  • abnormal vibrissa morphology / IMPC
  • preweaning lethality, incomplete penetrance / IMPC
MGI phenotypes (allele matching)
  • abnormal cochlea morphology / MGI
  • circling / MGI
  • abnormal posture / MGI
  • impaired swimming / MGI
  • impaired righting response / MGI
  • impaired balance / MGI
  • abnormal semicircular canal morphology / MGI
  • detached otolithic membrane / MGI
  • abnormal tectorial membrane morphology / MGI
  • abnormal crista ampullaris morphology / MGI
  • head tilt / MGI
  • abnormal vestibular saccule morphology / MGI
  • abnormal utricle morphology / MGI
  • impaired hearing / MGI
  • absent pinna reflex / MGI
  • increased or absent threshold for auditory brainstem response / MGI
MGI phenotypes (gene matching)
  • abnormal cochlea morphology / MGI
  • circling / MGI
  • hyperactivity / MGI
  • impaired coordination / MGI
  • abnormal pinna reflex / MGI
  • abnormal posture / MGI
  • impaired swimming / MGI
  • impaired righting response / MGI
  • impaired balance / MGI
  • abnormal placing response / MGI
  • reduced fertility / MGI
  • deafness / MGI
  • abnormal semicircular canal morphology / MGI
  • abnormal otolith morphology / MGI
  • abnormal otolithic membrane morphology / MGI
  • detached otolithic membrane / MGI
  • abnormal tectorial membrane morphology / MGI
  • abnormal ear physiology / MGI
  • abnormal crista ampullaris morphology / MGI
  • abnormal vestibular saccular macula morphology / MGI
  • decreased vestibular hair cell stereocilia number / MGI
  • head tilt / MGI
  • abnormal vestibular saccule morphology / MGI
  • abnormal utricle morphology / MGI
  • impaired hearing / MGI
  • nonsyndromic hearing impairment / MGI
  • absent pinna reflex / MGI
  • absent startle reflex / MGI
  • increased or absent threshold for auditory brainstem response / MGI

Literature references

  • Twister mutant mice are defective for otogelin, a component specific to inner ear acellular membranes;Simmler MC, Zwaenepoel I, Verpy E, Guillaud L, Elbaz C, Petit C, Panthier JJ;2000;Mammalian genome : official journal of the International Mammalian Genome Society;11;960-6; 11063250
  • Targeted disruption of otog results in deafness and severe imbalance.;Simmler M C, Cohen-Salmon M, El-Amraoui A, Guillaud L, Benichou J C, Petit C, Panthier J J, ;2000;Nature genetics;24;139-43; 10655058

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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

Example health report
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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.

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