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.
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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