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Progressive rod-cone degeneration

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Full-Text Articles in Medicine and Health Sciences

Phenotypic Characterizations And Genetic Study Of Progressive Rod-Cone Degeneration In Poodles In Thailand, Metita Sussadee, Janjira Phavaphutanon, Chaiyan Kasorndorkbua, Areerat Kongcharoen, Preenun Jitasombuti, Sudtisa Laopiem, Aree Thayananuphat Jun 2014

Phenotypic Characterizations And Genetic Study Of Progressive Rod-Cone Degeneration In Poodles In Thailand, Metita Sussadee, Janjira Phavaphutanon, Chaiyan Kasorndorkbua, Areerat Kongcharoen, Preenun Jitasombuti, Sudtisa Laopiem, Aree Thayananuphat

The Thai Journal of Veterinary Medicine

Progressive rod-cone degeneration (PRCD) is one of several groups of inherited progressive retinal atrophy in dogs. This study aims to describe the characterization of phenotypic appearance and demonstrate the responsible gene mutation, including analysis of the allele frequency of PRCD in Poodle dogs in Thailand. All 10 Poodles with clinical signs of PRCD were identified by a history of progressive vision loss, an abnormal obstacle test, and fundic appearance. Genetic testing of PRCD gene mutation using the Polymerase Chain Reaction and Restriction Fragment Length Polymorphism technique was performed in all dogs. The result confirmed that all 10 dogs were affected. …

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Differential Expression Of Photoreceptor-Specific Proteins During Disease And Degeneration In The Progressive Rod-Cone Degeneration (Prcd) Retina, Kathryn Gropp, Jun Huang, Gustavo Aguirre May 1997

Differential Expression Of Photoreceptor-Specific Proteins During Disease And Degeneration In The Progressive Rod-Cone Degeneration (Prcd) Retina, Kathryn Gropp, Jun Huang, Gustavo Aguirre

Gustavo D. Aguirre, VMD, PhD

Progressive rod-cone degeneration (prcd) is a late-onset hereditary retinal degeneration characterized by normal development of photoreceptors prior to degeneration and death of visual cells. We reported previously that expression of opsin mRNA and protein decreases prior to visual cell degeneration. To examine the specificity of this reduction, we have used immunocytochemistry to correlate photoreceptor-specific protein expression with visual cell disease progression. Eyes from light-adapted age-matched control andprcd-affected dogs were fixed in paraformaldehyde, embedded in diethylene glycol distearate (DGD) wax, and reacted with antibodies specific to interphotoreceptor retinoid-binding protein (IRBP), S-antigen, opsin, phosducin, γ-phosphodiesterase (γ-PDE), and β1-transducin. While IRBP expression did …

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Canine Rod Transducin A-1: Cloning Of The Cdna And Evaluation Of The Gene As A Candidate For Progressive Retinal Atrophy, Kunal Ray, Victoria J. Baldwin, Caroline Zeiss, Gregory M. Acland, Gustavo D. Aguirre Dec 1996

Canine Rod Transducin A-1: Cloning Of The Cdna And Evaluation Of The Gene As A Candidate For Progressive Retinal Atrophy, Kunal Ray, Victoria J. Baldwin, Caroline Zeiss, Gregory M. Acland, Gustavo D. Aguirre

Gustavo D. Aguirre, VMD, PhD

Purpose: Progressive retinal atrophy (PRA) represents a heterogeneous group of retinal dystrophies, distinct forms of which occur in different canine breeds. The present study was undertaken to evaluate the gene for the a-1 subunit of the rod specific G-protein transducin (GNAT1), a member of the phototransduction pathway, as a candidate for progressive rod cone degeneration (prcd) in poodles, early retinal degeneration (erd) in elkhounds, and rod cone dysplasia 2 (rcd 2) in collies. 
Methods: Oligonucleotide primers were designed from the consensus region of known cDNA sequences for GNAT1 from other species. Canine GNAT1 cDNA was cloned and sequenced after reverse …

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Interphotoreceptor Retinoid-Binding Protein (Irbp) In Progressive Rod-Cone Degeneration (Prcd)—Biochemical, Immunocytochemical And Immunologic Studies, Gustavo Aguirre, Barbara Wiggert, Geetha Kutty, Kenneth Long, Lila Inouye, Igal Gery, Gerald Chader Aug 1991

Interphotoreceptor Retinoid-Binding Protein (Irbp) In Progressive Rod-Cone Degeneration (Prcd)—Biochemical, Immunocytochemical And Immunologic Studies, Gustavo Aguirre, Barbara Wiggert, Geetha Kutty, Kenneth Long, Lila Inouye, Igal Gery, Gerald Chader

Gustavo D. Aguirre, VMD, PhD

Interphotoreceptor retinoid-binding protein (IRBP) is synthesized and secreted by photoreceptor cells and is thought to facilitate the transport of retinoids during the visual cycle as well as fatty acids essential to the maintenance of normal outer segment membranes. Proteins such as IRBP, which are unique to the photoreceptor cells in the retina, are prime candidates in the consideration of biochemical defects which could contribute to photoreceptor cell degeneration in man and animals. In this study, the association between IRBP and retinal degeneration was examined using the progressive rod-cone degeneration (prcd) mutant retina in dogs as an animal model.
This study …

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The Cone Matrix Sheath In The Normal And Diseased Retina: Cytochemical And Biochemical Studies Of Peanut Agglutinin-Binding Proteins In Cone And Rod-Cone Degeneration, Kenneth Long, Gustavo Aguirre May 1991

The Cone Matrix Sheath In The Normal And Diseased Retina: Cytochemical And Biochemical Studies Of Peanut Agglutinin-Binding Proteins In Cone And Rod-Cone Degeneration, Kenneth Long, Gustavo Aguirre

Gustavo D. Aguirre, VMD, PhD

The fate of the cone-associated extracellular domain, or cone matrix sheath (CMS), was examined in two canine models of hereditary retinal degeneration. The diseases, which affect cones selectively (cd = cone degeneration), or rods and cones temporally (prcd = progressive rod-cone degeneration), were examined biochemically (SDS-PAGE/lectinblots) and cytochemically (light microscopy) using peanut agglutinin lectin (PNA) to selectively label this domain and associated structures. Most of the cones had disappeared in the adult cd retina. In the remaining cones, PNA labeled the ectopically located somata and the CMSs that were present around severely diseased ones. …

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