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Phenotypes Associated with This Genotype
Genotype
MGI:3826852
Allelic
Composition
Bsndtm1Tjj/Bsndtm1Tjj
Tg(Sox10-cre)1Wdr/0
Genetic
Background
involves: 129/Sv * 129X1/SvJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bsndtm1Tjj mutation (0 available); any Bsnd mutation (11 available)
Tg(Sox10-cre)1Wdr mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• mice exhibit abnormal gain and phase of angular vestibulo ocular reflexes (VOR) in the dark compared to wild-type mice
• however, VORs in the light are normal
• after 3 weeks, outer hair cells in the basal turn begin to degenerate
• as the stria vascularis degenerates it loses the normal interdigitation of marginal and intermediate cells
• however, all cell types are retained
• at 2 weeks, the width of the stria vascularis is reduced by 30% to 50% compared to in wild-type mice
• between P20 and P30
• at 3 weeks, mice exhibit hearing loss of about 60 dB as measured by auditory brainstem response that is less severe than in Slc12a2tm2Bhk homozygotes

behavior/neurological
• mice exhibit abnormal gain and phase of angular vestibulo ocular reflexes (VOR) in the dark compared to wild-type mice
• however, VORs in the light are normal

nervous system
• after 3 weeks, outer hair cells in the basal turn begin to degenerate

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Bartter disease type 4a DOID:0110145 OMIM:602522
J:143314


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
02/11/2020
MGI 6.14
The Jackson Laboratory