STOCK Srrm4bv/WtsiH
Status | Available to order |
EMMA ID | EM:01315 |
Citation information | RRID:IMSR_EM:01315 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information. |
International strain name | STOCK Srrm4bv/WtsiH |
Alternative name | bv, Bronx Waltzer |
Strain type | Spontaneous |
Allele/Transgene symbol | Srrm4bv |
Gene/Transgene symbol | Srrm4 |
Information from provider
Provider | Karen Steel |
Provider affiliation | Wellcome Trust Sanger Institute |
Genetic information | This is a spontaneous mutation affecting the sensory hair cells of the inner ear and leading to deafness and balance defects with circling and head bobbing. |
Phenotypic information | This is a hearing impaired, recessive mutant that has a primary defect in the sensory cells of the organ of Corti of the inner ear. Homozygotes show circling and head bobbing and often do not show a Preyer reflex. Heterozygotes and wildtypes are not distinguishable from each other. |
Breeding history | Originated on an unknown genetic background and maintained on same background within a closed colony for over 30 years. |
References |
|
Homozygous fertile | yes |
Homozygous viable | yes |
Homozygous matings required | no |
Immunocompromised | no |
Information from EMMA
Archiving centre | Mary Lyon Centre at MRC Harwell, Oxford, United Kingdom |
Disease and phenotype information
IMPC phenotypes (gene matching)
MGI phenotypes (allele matching)
- abnormal inner ear morphology / MGI
- circling / MGI
- abnormal pinna reflex / MGI
- impaired balance / MGI
- deafness / MGI
- abnormal vestibular hair cell morphology / MGI
- cochlear ganglion degeneration / MGI
- abnormal pillar cell morphology / MGI
- abnormal crista ampullaris morphology / MGI
- absent pillar cells / MGI
- decreased vestibular hair cell number / MGI
- abnormal utricular macula morphology / MGI
- utricular macular degeneration / MGI
- abnormal cochlear inner hair cell morphology / MGI
- decreased cochlear inner hair cell number / MGI
- absent cochlear inner hair cells / MGI
- cochlear inner hair cell degeneration / MGI
- absent vestibular hair cell stereocilia / MGI
- abnormal cochlear hair cell development / MGI
- abnormal distortion product otoacoustic emission / MGI
- head tossing / MGI
- abnormal hearing electrophysiology / MGI
- abnormal otoacoustic response / MGI
- increased or absent threshold for auditory brainstem response / MGI
MGI phenotypes (gene matching)
- abnormal inner ear morphology / MGI
- tremors / MGI
- abnormal corpus callosum morphology / MGI
- abnormal stratification in cerebral cortex / MGI
- circling / MGI
- abnormal pinna reflex / MGI
- impaired balance / MGI
- cyanosis / MGI
- respiratory distress / MGI
- deafness / MGI
- abnormal innervation / MGI
- abnormal vestibular hair cell morphology / MGI
- cochlear ganglion degeneration / MGI
- abnormal axon guidance / MGI
- no phenotypic analysis / MGI
- premature neuronal precursor differentiation / MGI
- abnormal axon extension / MGI
- abnormal pillar cell morphology / MGI
- abnormal crista ampullaris morphology / MGI
- abnormal embryonic/fetal subventricular zone morphology / MGI
- absent pillar cells / MGI
- decreased vestibular hair cell number / MGI
- abnormal utricular macula morphology / MGI
- utricular macular degeneration / MGI
- abnormal cochlear inner hair cell morphology / MGI
- decreased cochlear inner hair cell number / MGI
- absent cochlear inner hair cells / MGI
- cochlear inner hair cell degeneration / MGI
- absent vestibular hair cell stereocilia / MGI
- abnormal cochlear hair cell development / MGI
- abnormal distortion product otoacoustic emission / MGI
- decreased neuronal precursor cell number / MGI
- head tilt / MGI
- head tossing / MGI
- abnormal hearing electrophysiology / MGI
- abnormal otoacoustic response / MGI
- abnormal neurite morphology / MGI
- decreased survivor rate / MGI
- decreased neuron number / MGI
- neonatal lethality, incomplete penetrance / MGI
- increased or absent threshold for auditory brainstem response / MGI
- abnormal phrenic nerve innervation pattern to diaphragm / MGI
Literature references
- Localization of the bronx waltzer (bv) deafness gene to mouse chromosome 5.;Bussoli T J, Kelly A, Steel K P, ;1997;Mammalian genome : official journal of the International Mammalian Genome Society;8;714-7; 9321462
- The inner ear in Bronx waltzer mice.;Deol M S, ;1981;Acta oto-laryngologica;92;331-6; 7324900
- Early development and degeneration of vestibular hair cells in bronx waltzer mutant mice.;Cheong Michael A, Steel Karen P, ;2002;Hearing research;164;179-89; 11950537
- Characteristic findings of auditory brainstem response and otoacoustic emission in the Bronx waltzer mouse.;Inagaki Masumi, Kon Kaori, Suzuki Seiko, Kobayashi Naoko, Kaga Makiko, Nanba Eiji, ;2006;Brain & development;28;617-24; 16730938
- Influence of neurotrophins on the synaptogenesis of inner hair cells in the deaf Bronx waltzer (bv) mouse organ of Corti in culture.;Sobkowicz Hanna M, August Benjamin K, Slapnick Susan M, ;2002;International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience;20;537-54; 12485622
- Cochlear inner hair cells exist transiently in the fetal Bronx Waltzer (bv/bv) mouse.;Whitlon D S, Gabel C, Zhang X, ;1996;The Journal of comparative neurology;364;515-522; 8820880
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