Abstract

Specific strains of dogs, affected with well-characterized hereditary retinal degenerations (Irish setter: Rod-Cone Dysplasia, miniature poodle: Progressive Rod-Cone Degeneration, Norwegian elkhound: Early Retinal Degeneration, Alaskan malamute: Hemeralopial, together with appropriate nonaffected control dogs, will be bred and maintained. The progeny of these dogs will be distributed to approved research investigators, either directly or by collection, processing and distribution of requested tissues. The primary aims in breeding setters, poodles and elkhounds are to ensure the continued availability of their respective mutations and to promote their utilization by research investigators. The primary aim in breeding malamutes is to preserve this unique mutation and ensure its future availability. The utilization of these mutants will be promoted by solicitation of requests from investigators. Responses will be reviewed for scientific merit and prioritized by an independent Animal Distribution Committee (ADC). The allocation and distribution of dogs and/or tissues will be according to the priorities set by the ADC. Assistance will be offered to investigators in development and implementation of specific protocols for optimal utilization of these mutants.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Eye Institute (NEI)
Type
Research Project--Cooperative Agreements (U01)
Project #
5U01EY006855-04
Application #
3551964
Study Section
Vision Research and Training Committee (VSN)
Project Start
1986-09-30
Project End
1991-09-29
Budget Start
1989-09-30
Budget End
1990-09-29
Support Year
4
Fiscal Year
1989
Total Cost
Indirect Cost

  Institution

Name
University of Pennsylvania
Department
Type
Schools of Veterinary Medicine
DUNS #
042250712
City
Philadelphia
State
PA
Country
United States
Zip Code
19104

  Publications

Gardiner, Kristin L; Downs, Louise; Berta-Antalics, Agnes I et al. (2016) Photoreceptor proliferation and dysregulation of cell cycle genes in early onset inherited retinal degenerations. BMC Genomics 17:221
Marsili, Stefania; Genini, Sem; Sudharsan, Raghavi et al. (2015) Exclusion of the unfolded protein response in light-induced retinal degeneration in the canine T4R RHO model of autosomal dominant retinitis pigmentosa. PLoS One 10:e0115723
Genini, Sem; Beltran, William A; Aguirre, Gustavo D (2013) Up-regulation of tumor necrosis factor superfamily genes in early phases of photoreceptor degeneration. PLoS One 8:e85408
Komáromy, András M; Rowlan, Jessica S; Corr, Amanda T Parton et al. (2013) Transient photoreceptor deconstruction by CNTF enhances rAAV-mediated cone functional rescue in late stage CNGB3-achromatopsia. Mol Ther 21:1131-41
Kun, Teri; Lyons, Leslie A; Sacks, Benjamin N et al. (2013) Developmental validation of Mini-DogFiler for degraded canine DNA. Forensic Sci Int Genet 7:151-8
Guziewicz, Karina E; Zangerl, Barbara; Komáromy, András M et al. (2013) Recombinant AAV-mediated BEST1 transfer to the retinal pigment epithelium: analysis of serotype-dependent retinal effects. PLoS One 8:e75666
Beltran, William A; Cideciyan, Artur V; Lewin, Alfred S et al. (2012) Gene therapy rescues photoreceptor blindness in dogs and paves the way for treating human X-linked retinitis pigmentosa. Proc Natl Acad Sci U S A 109:2132-7
Guziewicz, Karina E; Aguirre, Gustavo D; Zangerl, Barbara (2012) Modeling the structural consequences of BEST1 missense mutations. Adv Exp Med Biol 723:611-8
Kukekova, Anna V; Johnson, Jennifer L; Teiling, Clotilde et al. (2011) Sequence comparison of prefrontal cortical brain transcriptome from a tame and an aggressive silver fox (Vulpes vulpes). BMC Genomics 12:482
Guziewicz, Karina E; Slavik, Julianna; Lindauer, Sarah J P et al. (2011) Molecular consequences of BEST1 gene mutations in canine multifocal retinopathy predict functional implications for human bestrophinopathies. Invest Ophthalmol Vis Sci 52:4497-505

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