Search Criteria: Research Area is "Sensorineural Research: Retinal Degeneration (retinitis pigmentosa)"
| Stock Number |
Strain Name Strain Description |
Standard Supply |
| 004510 | STOCK Rom1tm1Mci/J | Repository- Live |
| Mice that are homozygous for the targeted mutation are viable and normal in size. When heterozygous mice are bred together, homozygous animals occur at a greatly reduced frequency (~6%). No gene product (protein) is detected in retinal tissue from homozygotes by Western blot analysis. Onset of progressive retinal degeneration occurs at 2 months of age beginning with a thinning of the outer nuclear layer of retinal cells. Rod outer segments in 2 month old mice display disorganized arrangement, irregular gaps and amorphous aggregates. At 4 months of age organization of rod outer segments improves. TUNEL assay of mutant retinal tissue show photoreceptor degeneration is due to apoptotic cell death. Ultra structural organization of rod outer segment disks is disorganized, often with patches of enlarged disks. Electroretinogram a-wave analysis of photoreceptor function reveals a diminished maximal photoreceptor response (50% lower than wildtype). This mutant mouse strain may be useful in stu
..... For more information please see the full descriiption on the strain data sheet | ||
| 007064 | B6.129-Crxtm1Clc/J | Repository-Cryopreserved |
| Homozygotes are viable and fertile and do not display any gross physical abnormalities. These mice do not elaborate photoreceptor outer segments and synaptic termini, and they lack cone and rod activities as assayed by electroretinograms. Circadian entrainment activity is attenuated. Protein is not produced as determined by RT-PCR of retinal RNA extracts. This strain may be useful as a model of retinophathy (e.g., Leber's congenital amaurosis (LCA), cone-rod dystrophy-2 (adCRD2), retinitis pigmentosa (RP)), or circadian rhythm modification. | ||
| 004953 | B6;129S6-Gucy2etm1Gar/J | Repository-Cryopreserved |
| Mice that are homozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No gene product (protein) is detected by Western blot analysis of eye tissue. Progressive loss of electroretinograms (ERGs) of cone cells from homozygotes begins at age 3 to 5 weeks. ERGs are completely lost in mice older than 8 weeks of age. Histological analysis of mutants between 4 and 5 weeks of age reveals retinal cone cell atrophy and a reduction in the number of cone cells. Isolated retinal rod cells exhibit reduced a-wave and b-wave ERGs, and impaired photoresponse. This mutant mouse strain may be useful in studies of incomplete achromatopsia, progressive cone-rod dystrophy, retinitis pigmentosa and Leber congenital amaurosis. | ||
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