Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pde6batrd1 mutation
(2 available);
any
Pde6b mutation
(119 available)
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vision/eye
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• pale by 10 weeks of age
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• slightly thinner at 3.5 weeks of age followed by rapid progressive thinning over the next 3 weeks
• at 6 weeks of age, 3-4 cell layers are present in the peripheral retina
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• slow onset of degeneration; the visual tracking response persists until several weeks of age in contrast to Pde6brd1 homozygous mice
(J:75964)
• develops as mice age, onset is later than in Pde6b rd1
(J:101336)
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• at 3.5 weeks of age, at least 20% of mice respond to a 2 degree grating and 90% respond to a 4 degree grating in a visual tracking drum assay
• at 6 weeks of age mice respond well to a 4 or 8 degree grating in a visual tracking drum assay
• by 10 weeks of age mice fail to respond in a visual tracking drum assay
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• effectively blind by 10 weeks of age
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nervous system
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• pale by 10 weeks of age
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Allelic
Composition |
Pde6batrd1/Pde6btm1Eye
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Genetic
Background |
involves: 129S4/SvJae * 129S6/SvEvTac * BALB/cAnN * C3H/HeN * C57BL/6J |
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pde6batrd1 mutation
(2 available);
any
Pde6b mutation
(119 available)
Pde6btm1Eye mutation
(0 available);
any
Pde6b mutation
(119 available)
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vision/eye
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• at 9 weeks of age small hyper-autofluorescence spots are distributed uniformly over the fundus
• at 18 weeks of age hyper-autofluorescence spots form larger patches of heterogenous size
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• progressive arteriolar narrowing is seen between 9 and 18 weeks of age
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• progressive reduction in the number of photoreceptors in the outer nuclear layer from 2 to 18 weeks of age
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• slow progressive photoreceptor degeneration
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• mixed rod-cone responses are lower and progressively decrease over time
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• decreases in photopic cone responses are slower than those in rod or mixed rod-cone responses
• decreases are seen after 15 weeks of age
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• rod-specific b-wave peaks are depressed or extinguished in mice between 6 and 18 weeks of age
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nervous system
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• progressive reduction in the number of photoreceptors in the outer nuclear layer from 2 to 18 weeks of age
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• slow progressive photoreceptor degeneration
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cardiovascular system
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• progressive arteriolar narrowing is seen between 9 and 18 weeks of age
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vision/eye
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• patches of pigmented cells are seen at 3.5 weeks of age
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• slow onset of degeneration; the visual tracking response persists until several weeks of age in contrast to Pde6brd1 homozygous mice
(J:75964)
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• at 3.5 weeks of age, no mice respond to a 2 degree grating in a visual tracking drum assay
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vision/eye
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N |
• treatment with tamoxifen at E0.5 or E12 prevents the progressive photoreceptor degeneration seen in heterozygous mice without cre expression
• treatment with tamoxifen at E0.5 also rescues the hyper-autofluorescence fundus phenotype
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• following tamoxifen treatment at E12 or P1and P2, at 18 weeks of age hyper-autofluorescence spots of heterogeneous size and non-uniform distribution are seen on the fundus
• tamoxifen treatment at P1 fails to rescue the fundus hyper-autofluorescence phenotype
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• tamoxifen treatment at E12 or P1 and P2 results in partial rescue of progressive arteriolar narrowing at 18 weeks of age
• tamoxifen treatment at E0.5 results in complete rescue of arteriolar narrowing at 18 weeks of age
• tamoxifen treatment at P1 fails to rescue arteriolar narrowing
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• only treatment with tamoxifen at E0.5 resulted in photoreceptor numbers similar to controls after P18
• mice treated with tamoxifen at E12 or P1 have fewer photoreceptors by 18 weeks of age compared to controls
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• cones show a distinct trailing edge/ring-shaped shape, reminiscent of a flame instead of the normal small, round, punctate shape at E12
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• treatment with a single dose of tamoxifen at P1 fails to prevent progressive retinal photoreceptor degeneration
• treatment with 2 doses of tamoxifen at P1 and P2 partially rescues the progressive retinal photoreceptor degeneration
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nervous system
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• only treatment with tamoxifen at E0.5 resulted in photoreceptor numbers similar to controls after P18
• mice treated with tamoxifen at E12 or P1 have fewer photoreceptors by 18 weeks of age compared to controls
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• cones show a distinct trailing edge/ring-shaped shape, reminiscent of a flame instead of the normal small, round, punctate shape at E12
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• treatment with a single dose of tamoxifen at P1 fails to prevent progressive retinal photoreceptor degeneration
• treatment with 2 doses of tamoxifen at P1 and P2 partially rescues the progressive retinal photoreceptor degeneration
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cardiovascular system
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• tamoxifen treatment at E12 or P1 and P2 results in partial rescue of progressive arteriolar narrowing at 18 weeks of age
• tamoxifen treatment at E0.5 results in complete rescue of arteriolar narrowing at 18 weeks of age
• tamoxifen treatment at P1 fails to rescue arteriolar narrowing
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Analysis Tools