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 …
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. …
Glycosaminoglycan And Collagen Metabolism In Arylsulfatase B-Deficient Retinal Pigment Epithelium In Vitro, Gustavo D. Aguirre, Lawrence E. Stramm, W Li, M Haskins
May 1991
Glycosaminoglycan And Collagen Metabolism In Arylsulfatase B-Deficient Retinal Pigment Epithelium In Vitro, Gustavo D. Aguirre, Lawrence E. Stramm, W Li, M Haskins
Gustavo D. Aguirre, VMD, PhD
Regional differences in retinal pigment epithelial (RPE) cell glycosaminoglycan (GAG) and collagen metabolism were studied using cells obtained from normal cats and those with deficient activity of arylsulfatase B (ASB), a lysosomal enzyme involved in GAG catabolism. Control and ASB-deficient RPE cultures initiated from superior equatorial (superior) and inferior equatorial (inferior) regions of the eye were radiolabeled for 72 hr with 35SO4, and GAGs from the media and cell layers were analyzed separately. In ASB-deficient RPE, there was an accumulation of dermatan/chondroitin sulfate in the cell layer of cultures initiated from the superior region of the eye but not in …