C57BL/6N-Cdh23^{tm2b(EUCOMM)Wtsi}/WtsiH

Status	Available to order
EMMA ID	EM:14252
International strain name	C57BL/6N-Cdh23^{tm2b(EUCOMM)Wtsi}/WtsiH
Alternative name	MEPD1008_2_A03
Strain type	Targeted Mutant Strains
Allele/Transgene symbol	Cdh23^{tm2b(EUCOMM)Wtsi}
Gene/Transgene symbol	Cdh23
Disclaimer	Please note that for EUCOMM and KOMP-CSD mice supplied to the scientific community by INFRAFRONTIER/EMMA: We can not guarantee a null mutation for Knock-out first alleles (tm1a alleles, see http://www.mousephenotype.org/about-ikmc/targeting-strategies) as the critical exon has not been deleted. That the structure of the targeted mutation in the ES cells obtained from EUCOMM/KOMP to generate EUCOMM/KOMP mice is not verified by INFRAFRONTIER/EMMA. It is recommended that the recipient confirms the mutation structure. No check for determining the copy number of the targeting construct in ES cells obtained from EUCOMM/KOMP is done by INFRAFRONTIER/EMMA. The level of quality control before mice are released is to confirm the individual mouse genotype by short range PCR.

Information from provider

Provider	Wellcome Trust Sanger Institute
Provider affiliation	Wellcome Trust Sanger Institute
Genetic information	This mouse line originates from EUCOMM ES clone MEPD1008_2_A03. For further details on the construction of this clone see the page at the IMPC portal. The critical exon(s) were flanked by loxP sites, and subsequent cre expression excised this critical sequence resulting in a knockout reporter allele using a cell permeable HTN-Cre as described in doi:10.1007/s11248-013-9764-x. Click here for more information on EUCOMM final vectors.
Phenotypic information	Potential phenotyping data in the IMPC portal
References	None available

Information from EMMA

Archiving centre	Mary Lyon Centre at MRC Harwell, Oxford, United Kingdom

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Usher syndrome type 1 / Orphanet_231169
Autosomal recessive non-syndromic sensorineural deafness type DFNB / Orphanet_90636

IMPC phenotypes (allele matching)

decreased circulating calcium level / IMPC
decreased leukocyte cell number / IMPC
decreased body length / IMPC
abnormal gait / IMPC
stereotypic behavior / IMPC
trunk curl / IMPC
impaired righting response / IMPC
increased circulating chloride level / IMPC
increased lean body mass / IMPC
decreased circulating glucose level / IMPC
increased mean corpuscular hemoglobin / IMPC
increased circulating sodium level / IMPC
absent pinna reflex / IMPC
decreased total body fat amount / IMPC
increased bone mineral content / IMPC
preweaning lethality, incomplete penetrance / IMPC
decreased fasting circulating glucose level / IMPC

IMPC phenotypes (gene matching)

decreased circulating calcium level / IMPC
preweaning lethality, incomplete penetrance / IMPC
absent pinna reflex / IMPC
decreased body length / IMPC
decreased circulating glucose level / IMPC
increased mean corpuscular hemoglobin / IMPC
trunk curl / IMPC
increased lean body mass / IMPC
decreased total body fat amount / IMPC
increased circulating sodium level / IMPC
abnormal gait / IMPC
impaired righting response / IMPC
decreased fasting circulating glucose level / IMPC
increased circulating chloride level / IMPC
stereotypic behavior / IMPC
decreased leukocyte cell number / IMPC
increased bone mineral content / IMPC

MGI phenotypes (gene matching)

abnormal ear position / MGI
abnormal inner ear morphology / MGI
abnormal cochlea morphology / MGI
abnormal organ of Corti morphology / MGI
organ of Corti degeneration / MGI
abnormal stria vascularis morphology / MGI
tremors / MGI
abnormal maternal nurturing / MGI
abnormal stationary movement / MGI
abnormal locomotor behavior / MGI
ataxia / MGI
circling / MGI
bidirectional circling / MGI
hyperactivity / MGI
abnormal gait / MGI
head bobbing / MGI
decreased grooming behavior / MGI
abnormal startle reflex / MGI
decreased startle reflex / MGI
impaired swimming / MGI
decreased litter size / MGI
abnormal reflex / MGI
abnormal hearing physiology / MGI
deafness / MGI
abnormal motor capabilities/coordination/movement / MGI
abnormal ear morphology / MGI
abnormal cochlear hair cell morphology / MGI
head shaking / MGI
decreased vertical activity / MGI
abnormal cochlear ganglion morphology / MGI
cochlear ganglion degeneration / MGI
abnormal otolith morphology / MGI
abnormal response to novelty / MGI
abnormal cochlear sensory epithelium morphology / MGI
cochlear ganglion hypoplasia / MGI
abnormal defecation / MGI
abnormal ear physiology / MGI
vestibular saccular degeneration / MGI
vestibular saccular macula degeneration / MGI
cochlear hair cell degeneration / MGI
stria vascularis degeneration / MGI
decreased cochlear inner hair cell number / MGI
cochlear inner hair cell degeneration / MGI
abnormal cochlear outer hair cell morphology / MGI
decreased cochlear outer hair cell number / MGI
cochlear outer hair cell degeneration / MGI
decreased cochlear hair cell number / MGI
abnormal cochlear nerve compound action potential / MGI
abnormal hair cell mechanoelectric transduction / MGI
abnormal cochlear hair cell physiology / MGI
abnormal cochlear outer hair cell physiology / MGI
abnormal orientation of outer hair cell stereociliary bundles / MGI
abnormal orientation of inner hair cell stereociliary bundles / MGI
abnormal vestibular hair cell stereociliary bundle morphology / MGI
abnormal cochlear hair cell stereociliary bundle morphology / MGI
abnormal orientation of cochlear hair cell stereociliary bundles / MGI
decreased cochlear hair cell stereocilia number / MGI
abnormal outer hair cell stereociliary bundle morphology / MGI
decreased outer hair cell stereocilia number / MGI
absent outer hair cell stereocilia / MGI
abnormal inner hair cell stereociliary bundle morphology / MGI
abnormal cochlear hair cell inter-stereocilial links morphology / MGI
abnormal cochlear hair bundle tip links morphology / MGI
increased susceptibility to noise-induced hearing loss / MGI
abnormal distortion product otoacoustic emission / MGI
absent distortion product otoacoustic emissions / MGI
abnormal auditory brainstem response / MGI
abnormal vestibular system physiology / MGI
increased susceptibility to age-related hearing loss / MGI
absent linear vestibular evoked potential / MGI
head tilt / MGI
head tossing / MGI
novel environmental response-related retropulsion / MGI
homeostasis/metabolism phenotype / MGI
hearing/vestibular/ear phenotype / MGI
behavior/neurological phenotype / MGI
vision/eye phenotype / MGI
abnormal eye electrophysiology / MGI
impaired hearing / MGI
absent pinna reflex / MGI
absent startle reflex / MGI
abnormal inner hair cell kinocilium morphology / MGI
abnormal outer hair cell kinocilium morphology / MGI
increased or absent threshold for auditory brainstem response / MGI
decreased a wave implicit time / MGI
decreased b wave implicit time / MGI

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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 sperm. Delivered in 4 weeks (after paperwork in place). €1740^*
Tissue - Types of tissue, service fee and delivery time available upon request

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).

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C57BL/6N-Cdh23tm2b(EUCOMM)Wtsi/WtsiH