This application proposes continuance of the project """"""""Canine Models of Hereditary Retinal Degeneration"""""""", as a national research resource for studies of hereditary retinal degenerations. The need for these canine mutants in research studies is addressed, as is the importance of maintaining them in a centralized breeding and distribution facility. Examples of research studies that will utilize the colony, and their significance, are summarized. Specific and comprehensive details of such research are not included, however, as these form part of the peer reviewed research programs (funded by ROI or other support) of those individual investigators utilizing the colony's resources. Four specific mutant strains of dog, each transmitting a different, unique gene for a well-characterized, autosomal recessive hereditary retinal degeneration (rcd1: Rod-Cone Dysplasia Type 1; prcd: Progressive Rod-Cone Degeneration; erd: Early Retinal Degeneration; cd: Cone Degeneration) together with appropriate nonaffected control dogs, will be bred, maintained and made available for approved research investigations. The progeny of these dogs will be distributed to approved research investigators either directly or by collection, processing and distribution of requested tissues. DNA from representative individuals and from informative pedigrees of each mutant strain will be preserved and made available to all approved investigators. The primary aims of this project are to ensure continued availability of the respective mutations and to promote their utilization by research investigators. Utilization will be promoted by solicitation of requests from investigators. Responses will be reviewed for scientific merit and priorized by an independent Retinal Research Resources Committee (RRRC), appointed and chaired by appropriate NEI/NIH staff. Allocation of distributable resources (such as dogs, tissues, DNA) from the colony will be according to priorities set by the RRRC. Investigators will be assisted in the development and implementation of protocols for optimal utilization of these mutants.

Agency
National Institute of Health (NIH)
Institute
National Eye Institute (NEI)
Type
Research Project (R01)
Project #
2R01EY006855-06
Application #
3263533
Study Section
Visual Sciences C Study Section (VISC)
Project Start
1986-09-30
Project End
1992-11-30
Budget Start
1992-04-01
Budget End
1992-11-30
Support Year
6
Fiscal Year
1992
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
Guziewicz, Karina E; McTish, Emily; Dufour, Valerie L et al. (2018) Underdeveloped RPE Apical Domain Underlies Lesion Formation in Canine Bestrophinopathies. Adv Exp Med Biol 1074:309-315
Guziewicz, Karina E; Cideciyan, Artur V; Beltran, William A et al. (2018) BEST1 gene therapy corrects a diffuse retina-wide microdetachment modulated by light exposure. Proc Natl Acad Sci U S A 115:E2839-E2848
Cideciyan, Artur V; Sudharsan, Raghavi; Dufour, Valérie L et al. (2018) Mutation-independent rhodopsin gene therapy by knockdown and replacement with a single AAV vector. Proc Natl Acad Sci U S A 115:E8547-E8556
Miyadera, Keiko (2018) Mapping of Canine Models of Inherited Retinal Diseases. Adv Exp Med Biol 1074:257-264
Hardcastle, Alison J; Sieving, Paul A; Sahel, José-Alain et al. (2018) Translational Retinal Research and Therapies. Transl Vis Sci Technol 7:8
Sudharsan, Raghavi; Elliott, Michael H; Dolgova, Natalia et al. (2018) Photoreceptor Outer Segment Isolation from a Single Canine Retina for RPE Phagocytosis Assay. Adv Exp Med Biol 1074:593-601
Das, Rueben G; Marinho, Felipe Pompeo; Iwabe, Simone et al. (2017) Variabilities in retinal function and structure in a canine model of cone-rod dystrophy associated with RPGRIP1 support multigenic etiology. Sci Rep 7:12823
Yeh, Connie Y; Koehl, Kristin L; Harman, Christine D et al. (2017) Assessment of Rod, Cone, and Intrinsically Photosensitive Retinal Ganglion Cell Contributions to the Canine Chromatic Pupillary Response. Invest Ophthalmol Vis Sci 58:65-78
Sudharsan, Raghavi; Beiting, Daniel P; Aguirre, Gustavo D et al. (2017) Involvement of Innate Immune System in Late Stages of Inherited Photoreceptor Degeneration. Sci Rep 7:17897
Ye, Guo-Jie; Komáromy, András M; Zeiss, Caroline et al. (2017) Safety and Efficacy of AAV5 Vectors Expressing Human or Canine CNGB3 in CNGB3-Mutant Dogs. Hum Gene Ther Clin Dev 28:197-207

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