Abnormalities in outer segment renewal and/or cyclic nucleotide metabolism exist in rodents with hereditary retinal degeneration. Defects in these basic processes have been hypothesized to exist in other species, including man, affected with retinal degeneration. Developmental and degenerative forms of hereditary retinal degeneration are present in different breeds of dogs. In affected animals, the P.I. will determine if defective outer segment renewal and/or cyclic nucleotide metabolism are associated with the degeneration of the visual cells. Moreover, the P.I. will determine if these changes are the result of photoreceptor degeneration or cause the disease. Breeding experiments with affected dogs of different breeds will determine if single or multiple gene defects exist in dogs with hereditary retinal degeneration.

Agency
National Institute of Health (NIH)
Institute
National Eye Institute (NEI)
Type
Research Project (R01)
Project #
5R01EY001244-13
Application #
3255817
Study Section
Visual Sciences A Study Section (VISA)
Project Start
1979-03-01
Project End
1989-02-28
Budget Start
1985-03-01
Budget End
1986-02-28
Support Year
13
Fiscal Year
1985
Total Cost
Indirect Cost
Name
University of Pennsylvania
Department
Type
Schools of Veterinary Medicine
DUNS #
042250712
City
Philadelphia
State
PA
Country
United States
Zip Code
19104
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Gropp, K E; Huang, J C; Aguirre, G D (1997) Differential expression of photoreceptor-specific proteins during disease and degeneration in the progressive rod-cone degeneration (prcd) retina. Exp Eye Res 64:875-86
Aguirre, G D; Acland, G M; Maude, M B et al. (1997) Diets enriched in docosahexaenoic acid fail to correct progressive rod-cone degeneration (prcd) phenotype. Invest Ophthalmol Vis Sci 38:2387-407
Gropp, K E; Szel, A; Huang, J C et al. (1996) Selective absence of cone outer segment beta 3-transducin immunoreactivity in hereditary cone degeneration (cd). Exp Eye Res 63:285-96
Ray, K; Acland, G M; Aguirre, G D (1996) Nonallelism of erd and prcd and exclusion of the canine RDS/peripherin gene as a candidate for both retinal degeneration loci. Invest Ophthalmol Vis Sci 37:783-94
Ray, K; Trepanier, L A; Acland, G M et al. (1996) PCR/RFLP marker in the canine opsin gene. Anim Genet 27:293-4
Ray, K; Baldwin, V J; Acland, G M et al. (1995) Molecular diagnostic tests for ascertainment of genotype at the rod cone dysplasia 1 (rcd1) locus in Irish setters. Curr Eye Res 14:243-7

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