Search Criteria: Research Area is "Sensorineural Research: Retinal Degeneration: retinitis pigmentosa"
Additional Register Interest Strains
| Stock Number |
Strain Name Strain Description |
Standard Supply |
| 013148 | C57BL/6-Tg(Pdgfra-cre)1Clc/J | Repository- Live |
| Hemizygous Pdgfra-cre mice are viable and fertile, with cre expression directed to retinal Muller glial cells by the mouse Pdgfra (platelet derived growth factor receptor, alpha polypeptide) promoter. Expression is predominantly in the cell bodies of the inner nuclear layer (INL) of the retina, but some expression may be observed in the outer nuclear layer (ONL) and in the ganglion cell layer (GCL). The donating investigator indicates that although not examined, cre may also be active in many types of central nervous system glial cells. When bred with mice containing a loxP-flanked sequence of interest, Cre-mediated recombination will result in deletion of the floxed sequence(s) in the offspring. | ||
| 017557 | C57BL/6-Tg(BEST1-cre)1Jdun/J | Under Development - Now Accepting Orders |
| These mice express Cre recombinase under the control of the human bestrophin 1 (BEST) promoter. Mosaic Cre recombinase expression (mRNA) is seen in 50-90% of retinal pigment epithelium (RPE) cells, with some expression seen in the testis. When crossed with a strain containing a loxP site-flanked sequence, Cre-mediated recombination results in deletion of the flanked sequence in RPE cells. Homozygotes are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. This strain represents an effective tool for generating RPE specific-targeted mutants that would be useful in studies of human retinopathies.
For example, when bred to a strain expressing a floxed-Dicer1 gene (see Dicer1tm1Bdh Stock No. 006366 for example), this mutant mouse strain may be useful for studying age-related macular degeneration. | ||
| 007064 | B6.129-Crxtm1Clc/J | Cryopreserved - Ready for recovery |
| 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 | Cryopreserved - Ready for recovery |
| 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. | ||
| 012426 | CBACa.129S6(Cg)-Ush1ctm1Bkts/J | Cryopreserved - Ready for recovery |
| These mice contain a mutation designed to replace gene function of the endogenous mouse Ush1c gene with the human USH1C function. They also contain a mutation (216G->A) designed to retain the endogenous mouse Ush1c sequence. Mice with this mutation carry the same truncated USH1C mRNA transcript and splicing defect as found in the cochleae and retinas of Usher 1C patients. Mice homozygous for Ush1c216A are viable, fertile, and exhibit behavioral characteristics of deaf mice; they are hyperactive, display circling and head tossing behavior, and do not have a Preyer reflex. They also develop retinal degeneration, as evident by absent auditory-evoked brainstem responses and progressive loss of rod photoreceptors, providing a novel resource to study the disease mechanism and the development of therapies. | ||
| 004510 | STOCK Rom1tm1Mci/J | Cryopreserved - Ready for recovery |
| 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 phenotype on the strain data sheet | ||
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