C3HeB/FeJ-Mir96^Dmdo/IegWtsiCnbc

Status	Available to order
EMMA ID	EM:05010
International strain name	C3HeB/FeJ-Mir96^Dmdo/IegWtsiCnbc
Alternative name	Diminuendo, Mir96
Strain type	Induced Mutant Strains : Chemically-induced
Allele/Transgene symbol	Mir96^Dmdo
Gene/Transgene symbol	Mir96

Information from provider

Provider	Karen Steel
Provider affiliation	Wellcome Trust Sanger Institute
Additional owner	Prof. M. Hrabé de Angelis, Helmholtz Zentrum München, Neuherberg, Germany
Genetic information	ENU-induced mutation of Mir96.
Phenotypic information	Heterozygotes show progressive hearing loss and homozygotes have profound deafness from an early age with some balance defects.
Breeding history	Originated from ENU mutagenesis programme in Munich, Helmholtz Centrum, on a C3HeB/FeJ background and bred on the same background within a closed colony or backcrossed to C3HeB/FeJ ever since.
References	An ENU-induced mutation of miR-96 associated with progressive hearing loss in mice.;Lewis Morag A, Quint Elizabeth, Glazier Anne M, Fuchs Helmut, De Angelis Martin Hrabé, Langford Cordelia, van Dongen Stijn, Abreu-Goodger Cei, Piipari Matias, Redshaw Nick, Dalmay Tamas, Moreno-Pelayo Miguel Angel, Enright Anton J, Steel Karen P, ;2009;Nature genetics;41;614-8; 19363478
Homozygous fertile	no
Homozygous viable	yes
Homozygous matings required	no
Immunocompromised	no

Information from EMMA

Archiving centre	CNB-CSIC, Centro Nacional de Biotecnologia, Madrid, Spain

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Autosomal dominant non-syndromic sensorineural deafness type DFNA / Orphanet_90635

IMPC phenotypes (allele matching)

decreased bone mineral density / IMPC
decreased body length / IMPC
decreased body weight / IMPC
abnormal cornea morphology / IMPC
abnormal gait / IMPC
abnormal startle reflex / IMPC
trunk curl / IMPC
limb grasping / IMPC
increased thermal nociceptive threshold / IMPC
decreased heart weight / IMPC
abnormal bone mineralization / IMPC
increased lean body mass / IMPC
decreased lean body mass / IMPC
abnormal behavior / IMPC
increased total body fat amount / IMPC
increased bone mineral content / IMPC

IMPC phenotypes (gene matching)

abnormal startle reflex / IMPC
abnormal auditory brainstem response / IMPC
increased leukocyte cell number / IMPC
trunk curl / IMPC
decreased body length / IMPC
increased bone mineral content / IMPC
increased thermal nociceptive threshold / IMPC
abnormal gait / IMPC
impaired righting response / IMPC
abnormal behavior / IMPC
decreased body weight / IMPC
increased circulating iron level / IMPC
increased lean body mass / IMPC
decreased heart weight / IMPC
increased circulating amylase level / IMPC
decreased lean body mass / IMPC
abnormal bone mineralization / IMPC
stereotypic behavior / IMPC
increased total body fat amount / IMPC
decreased bone mineral density / IMPC
limb grasping / IMPC
abnormal cornea morphology / IMPC
absent pinna reflex / IMPC

MGI phenotypes (allele matching)

abnormal pinna reflex / MGI
cochlear hair cell degeneration / MGI
cochlear outer hair cell degeneration / MGI
decreased cochlear nerve compound action potential / MGI
fused vestibular hair cell stereocilia / MGI
abnormal cochlear hair cell stereociliary bundle morphology / MGI
impaired hearing / MGI
circling / MGI
abnormal gait / MGI
head bobbing / MGI
deafness / MGI
decreased cochlear hair cell number / MGI
absent cochlear nerve compound action potential / MGI
absent pinna reflex / MGI

MGI phenotypes (gene matching)

circling / MGI
abnormal gait / MGI
head bobbing / MGI
abnormal pinna reflex / MGI
deafness / MGI
no abnormal phenotype detected / MGI
cochlear hair cell degeneration / MGI
cochlear outer hair cell degeneration / MGI
decreased cochlear hair cell number / MGI
absent cochlear nerve compound action potential / MGI
decreased cochlear nerve compound action potential / MGI
fused vestibular hair cell stereocilia / MGI
abnormal cochlear hair cell stereociliary bundle morphology / MGI
impaired hearing / MGI
absent pinna reflex / MGI

Literature references

An ENU-induced mutation of miR-96 associated with progressive hearing loss in mice.;Lewis Morag A, Quint Elizabeth, Glazier Anne M, Fuchs Helmut, De Angelis Martin Hrabé, Langford Cordelia, van Dongen Stijn, Abreu-Goodger Cei, Piipari Matias, Redshaw Nick, Dalmay Tamas, Moreno-Pelayo Miguel Angel, Enright Anton J, Steel Karen P, ;2009;Nature genetics;41;614-8; 19363478

Information on how we integrate external resources can be found here

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

Other EMMA strains

Not found what you were looking for? Search here for other strains available from EMMA.

C3HeB/FeJ-Mir96Dmdo/IegWtsiCnbc