Search Criteria: Research Area is "Neurobiology Research: Channel and Transporter Defects (potassium)"
| Stock Number |
Strain Name Strain Description |
Standard Supply |
| 004200 | B6;CBACa Aw-J/A-Npr2cn-2J/J | Repository- Live |
| Mice carrying the Npr2cn-2J display mutation short thick femurs, round heads, disorganized growth plates, and relatively normal vertebrae; A one month old female exhibited the same phenotype as above but also had splayed ribs. | ||
| 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. | ||
| 005830 | B6.129P2-Kcnq2tm1Dgen/J | Repository-Cryopreserved |
| This targeted mutant was created and characterized by Deltagen, Inc. View phenotypic data developed by Deltagen. | ||
| 000247 | B6CBACa Aw-J/A-Kcnj6wv/J | Repository-Cryopreserved |
| Mice homozygous for the weaver spontaneous mutation (Kcnj6wv) are recognizable in the second postnatal week by their small size, instability of gait, weakness, and hypotonia. Many homozygous mutant mice die at weaning age, but some survive to adulthood, and females may breed. The cerebellum in homozygous mutants is very small, simple, and almost devoid of granule cells, which degenerate during the second week. Heterozygotes behave normally, but they have a smaller than normal cerebellum with a deficiency of granule cells, some of which fail to migrate into the internal granule layer and remain scattered in the molecular layer. Evidence from cultures of mutant and normal cerebellum show that granule cells of Kcnj6wv/Kcnj6wv and Kcnj6wv/+ mice have gene-dosage dependent abnormalities in morphology and cell behavior. Studies using homozygous weaver/wildtype chimeras indicate that the migration defect of granule cells
..... For more information please see the full descriiption on the strain data sheet | ||
| 004407 | C3H/HeJCrl-Kcnq1vtg-2J/J | Repository-Cryopreserved |
| Kcnq1vtg-2J homozygotes exhibit circling and head-tossing behavior. Both clickbox and acoustic brainstem response (ABR) testing revealed that they are deaf by 8 weeks of age, while age matched heterozygotes have good hearing. This mutation was shown to be an allele of Kcnq1 by complementation testing with the original vertigo mutation, Kcnq1vtg. | ||
| 003532 | C3HeB.129S7-Kcna1tm1Tem/J | Repository-Cryopreserved |
| Homozygous null mice display an epileptic phenotype (episodic eye blinking, twitching of vibrissae, forelimb paddling, arrested motion, hyperstartle response) beginning during the third postnatal week. Quantitative RNase protection analysis on brain tissue indicates that in heterozygous mice the Kcna1 transcript is reduced to approximately half that observed in wild-type littermates. No transcripts were detected in homozygous mice. Approximately 50% of the homozygous mice die between the third and fifth weeks of life. Mice surviving this period survive for varying periods, depending on genetic background; congenic C3HeB/FeJ mice usually die at 6-8 weeks; hybrid N:NIHS-BC mice have been maintained for over 12 months during which they continue to display spontaneous seizures. A similar phenotype with spontaneous seizures has been observed in mice having 129/Sv, C3HeB/FeJ, C57BL/6 X 129/Sv, and 129/Sv X N:NIHS-BC backgrounds. | ||
| 004820 | C57BL/6J-Kcne12J/J | Repository-Cryopreserved |
| View strain phenotype and additional information on the Neuroscience Mutagenesis Facility web page for Kcne12J entry. | ||
| 005748 | C57BL/6J-Kcnq1vtg-3J/J | Repository-Cryopreserved |
| View strain phenotype and additional information on the Neuroscience Mutagenesis Facility web page for Kcnq1vtg-3J entry. | ||
| 004587 | C57BL/6J-Szt1/FrkJ | Repository-Cryopreserved |
| View strain phenotype and additional information on the Neuroscience Mutagenesis Facility web page for Szt1 entry. | ||
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