Search Criteria: Research Area is "Neurobiology Research: Vestibular and Hearing Defects (Jervell and Lange-Nielsen (JLN) syndromes)"
| Stock Number |
Strain Name Strain Description |
Standard Supply |
| 003009 | 129-Kcne1tm1Sfh/J | Repository-Cryopreserved |
| This strain is characterized by inner ear defects with a phenotype similar to shaker and waltzer. There is loss of hair cells and many supporting cells in the organ of Corti as well as in all vestibular end organs. The time course of hair cell degeneration is different for each portion of the inner ear. Heart abnormalities of homozygous mutant mice included a longer QT interval at slow heart rates, a paradoxical shorter QT interval at fast heart rates, and an overall exacerbated QT-heart rate adaptation compared with wild-type controls. This mutant serves as a model for Jervell and Lange-Nielsen disease in which patients suffer cardian arrhythmias and profound bilateral deafness. | ||
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