C57BL/6N-Atm1Brd Espnspdz Slc35f2tm1a(EUCOMM)Wtsi/WtsiH[cc]
Status | Available to order |
EMMA ID | EM:13796 |
International strain name | C57BL/6N-Atm1Brd Espnspdz Slc35f2tm1a(EUCOMM)Wtsi/WtsiH[cc] |
Alternative name | Spindizzy, Espn-spdz |
Strain type | Targeted Mutant Strains |
Allele/Transgene symbol | Slc35f2tm1a(EUCOMM)Wtsi, Espnspdz |
Gene/Transgene symbol | Slc35f2, Espn |
Information from provider
Provider | Karen Steel |
Provider affiliation | King |
Genetic information | We were unable to amplify a 431bp region in the intron between exons 15 and 16, between g.4:152122586 and g.4:152123017, suggesting that there may be an insertion or other genomic disruption at this location of the chromosome. |
Phenotypic information | Homozygous:Mice homozygous for this mutation displayed circling and head bobbing and had no response to sound up to 95dB SPL, associated with stereocilia defects of the inner ear hair cells.Heterozygous:No obvious defect. |
Breeding history | The spindizzy mutation arose in a colony carrying the Slc35f2tm1a(EUCOMM)Wtsi targeted mutation, and this allele may still be in the background. The spindizzy mutation was maintained within a closed colony on a C57BL/6N-Atm1Brd background over several years. |
References |
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Homozygous fertile | yes |
Homozygous viable | yes |
Homozygous matings required | no |
Immunocompromised | not known |
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
- Autosomal recessive non-syndromic sensorineural deafness type DFNB / Orphanet_90636
- Usher syndrome type 1 / Orphanet_231169
IMPC phenotypes (gene matching)
MGI phenotypes (gene matching)
- abnormal inner ear morphology / MGI
- abnormal inner ear vestibule morphology / MGI
- abnormal organ of Corti morphology / MGI
- organ of Corti degeneration / MGI
- abnormal stria vascularis morphology / MGI
- tremors / MGI
- abnormal nursing / MGI
- abnormal stationary movement / MGI
- abnormal locomotor behavior / MGI
- circling / MGI
- bidirectional circling / MGI
- abnormal gait / MGI
- decreased grooming behavior / MGI
- impaired swimming / MGI
- impaired balance / MGI
- abnormal reflex / MGI
- deafness / MGI
- abnormal cochlear hair cell morphology / MGI
- abnormal endocochlear potential / MGI
- head shaking / MGI
- decreased vertical activity / MGI
- abnormal cochlear ganglion morphology / MGI
- cochlear ganglion degeneration / MGI
- abnormal otolithic membrane morphology / MGI
- abnormal response to novelty / MGI
- abnormal tectorial membrane morphology / MGI
- detached tectorial membrane / MGI
- abnormal defecation / MGI
- abnormal ear physiology / MGI
- vestibular hair cell degeneration / MGI
- vestibular saccular macula degeneration / MGI
- abnormal utricular macula morphology / MGI
- cochlear hair cell degeneration / MGI
- cochlear inner hair cell degeneration / MGI
- cochlear outer hair cell degeneration / MGI
- abnormal crista ampullaris neuroepithelium morphology / MGI
- absent cochlear microphonics / MGI
- absent cochlear nerve compound action potential / MGI
- abnormal cochlear hair cell stereociliary bundle morphology / MGI
- decreased cochlear hair cell stereocilia number / MGI
- short cochlear hair cell stereocilia / MGI
- thin cochlear hair cell stereocilia / MGI
- abnormal inner hair cell stereociliary bundle morphology / MGI
- pillar cell degeneration / MGI
- abnormal vestibular system physiology / MGI
- absent linear vestibular evoked potential / MGI
- reduced linear vestibular evoked potential / MGI
- increased mean corpuscular hemoglobin / MGI
- increased hemoglobin content / MGI
- abnormal hearing electrophysiology / MGI
- absent pinna reflex / MGI
- retropulsion / MGI
- increased or absent threshold for auditory brainstem response / MGI
Literature references
- Identification and characterisation of spontaneous mutations causing deafness from a targeted knockout programme.;Lewis Morag A, Ingham Neil J, Chen Jing, Pearson Selina, Di Domenico Francesca, Rekhi Sohinder, Allen Rochelle, Drake Matthew, Willaert Annelore, Rook Victoria, Pass Johanna, Keane Thomas, Adams David J, Tucker Abigail S, White Jacqueline K, Steel Karen P, ;2022;BMC biology;20;67; 35296311
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