Click here to close
Hello! We notice that you are using Internet Explorer, which is not supported by Xenbase and may cause the site to display incorrectly.
We suggest using a current version of Chrome,
FireFox, or Safari.
???displayArticle.abstract???
Utilizing antisense oligonucleotides coupled with an intact Xenopus eye rudiment model, we have effectively demonstrated that we are able to downregulate the expression of a photoreceptor-specific protein, rds/peripherin, and generate a loss-of-function model upon which to further study the function of the rds/peripherin gene. The ultrastructure and protein expression patterns very closely resemble those previously documented in both the rds mouse and in human autosomal dominant retinitis pigmentosa due to peripherin/RDS mutations. An identical strategy can be applied to any gene correlated with a degenerative retinal phenotype. As the entire array of genes is revealed through the various genome projects, including human and mouse, it is becoming increasingly critical to evaluate and determine the function of the corresponding gene products. Discovering which gene is responsible for a particular clinical phenotype is only the first of many steps in the development of a treatment or cure for that particular disease. Using our in vitro model, in which the retina is readily accessible to the antisense oligonucleotide yet the normal three-dimensional ultrastructure of the retina is maintained, we can evaluate the function of virtually any gene as the sequence becomes available. A thorough understanding of the function of individual genes will provide insights on the role of gene product in retinal health and pathophysiology. This experimental approach will also allow for specific therapeutic interventions to be evaluated so that targeted treatments can be designed for individuals with specific genetic mutations.
Arikawa,
Localization of peripherin/rds in the disk membranes of cone and rod photoreceptors: relationship to disk membrane morphogenesis and retinal degeneration.
1992, Pubmed
Arikawa,
Localization of peripherin/rds in the disk membranes of cone and rod photoreceptors: relationship to disk membrane morphogenesis and retinal degeneration.
1992,
Pubmed
Bascom,
Cloning of the cDNA for a novel photoreceptor membrane protein (rom-1) identifies a disk rim protein family implicated in human retinopathies.
1992,
Pubmed
Besharse,
Membrane assembly in retinal photoreceptors. III. Distinct membrane domains of the connecting cilium of developing rods.
1985,
Pubmed
Besharse,
Membrane assembly in retinal photoreceptors I. Freeze-fracture analysis of cytoplasmic vesicles in relationship to disc assembly.
1980,
Pubmed
,
Xenbase
Brysch,
Design and application of antisense oligonucleotides in cell culture, in vivo, and as therapeutic agents.
1994,
Pubmed
Chong,
Repeated injections of a ciliary neurotrophic factor analogue leading to long-term photoreceptor survival in hereditary retinal degeneration.
1999,
Pubmed
Defoe,
Membrane assembly in retinal photoreceptors. II. Immunocytochemical analysis of freeze-fractured rod photoreceptor membranes using anti-opsin antibodies.
1985,
Pubmed
,
Xenbase
Denhardt,
Antisense strategies come of age. Antisense strategies sponsored by the New York Academy of Sciences, Philadelphia, PA, USA, January 12-15, 1992.
1992,
Pubmed
Faktorovich,
Photoreceptor degeneration in inherited retinal dystrophy delayed by basic fibroblast growth factor.
1990,
Pubmed
Flannery,
Degenerative changes in a retina affected with autosomal dominant retinitis pigmentosa.
1989,
Pubmed
Fliesler,
Membrane morphogenesis in retinal rod outer segments: inhibition by tunicamycin.
1985,
Pubmed
,
Xenbase
Harris,
Two cellular inductions involved in photoreceptor determination in the Xenopus retina.
1992,
Pubmed
,
Xenbase
Hawkins,
Development and degeneration of retina in rds mutant mice: photoreceptor abnormalities in the heterozygotes.
1985,
Pubmed
Hollyfield,
In vitro elimination of oocyte melanosomes from the retinal rudiment of the frog, Rana pipiens.
1976,
Pubmed
Hollyfield,
Pigmented retinal epithelium involvement in photoreceptor development and function.
1974,
Pubmed
Hollyfield,
Photoreceptor outer segment development: light and dark regulate the rate of membrane addition and loss.
1979,
Pubmed
,
Xenbase
Hollyfield,
Membrane addition to rod photoreceptor outer segments: light stimulates membrane assembly in the absence of increased membrane biosynthesis.
1982,
Pubmed
,
Xenbase
Hollyfield,
Membrane assembly in photoreceptor outer segments: progressive increase in 'open' basal discs with increased temperature.
1982,
Pubmed
,
Xenbase
Jablonski,
Pigment epithelium-derived factor supports normal development of photoreceptor neurons and opsin expression after retinal pigment epithelium removal.
2000,
Pubmed
,
Xenbase
Jablonski,
Lactose promotes organized photoreceptor outer segment assembly and preserves expression of photoreceptor proteins in retinal degeneration.
1999,
Pubmed
,
Xenbase
Jablonski,
Targeted disruption of Müller cell metabolism induces photoreceptor dysmorphogenesis.
2000,
Pubmed
,
Xenbase
Jablonski,
Closer look at lactose-mediated support of retinal morphogenesis.
2000,
Pubmed
,
Xenbase
Kedzierski,
Three homologs of rds/peripherin in Xenopus laevis photoreceptors that exhibit covalent and non-covalent interactions.
1996,
Pubmed
,
Xenbase
Kinney,
The photoreceptors and pigment epithelium of the larval Xenopus retina: morphogenesis and outer segment renewal.
1978,
Pubmed
,
Xenbase
Knox,
Transgene expression in Xenopus rods.
1998,
Pubmed
,
Xenbase
LaVail,
Protection of mouse photoreceptors by survival factors in retinal degenerations.
1998,
Pubmed
Linser,
Cell contact-dependent regulation of hormonal induction of glutamine synthetase in embryonic neural retina.
1982,
Pubmed
Linser,
Induction of glutamine synthetase in embryonic neural retina: localization in Müller fibers and dependence on cell interactions.
1979,
Pubmed
Lolley,
Cyclic GMP accumulation causes degeneration of photoreceptor cells: simulation of an inherited disease.
1977,
Pubmed
,
Xenbase
Prada,
Glutamine synthetase (GS) activity and spatial and temporal patterns of GS expression in the developing chick retina: relationship with synaptogenesis in the outer plexiform layer.
1998,
Pubmed
Röhlich,
Binding pattern of anti-rhodopsin monoclonal antibodies to photoreceptor cells: an immunocytochemical study.
1989,
Pubmed
Sheets,
Turning the frog into a princely model.
1998,
Pubmed
,
Xenbase
Stiemke,
Photoreceptor outer segment development in Xenopus laevis: influence of the pigment epithelium.
1994,
Pubmed
,
Xenbase
Stiemke,
Cell birthdays in Xenopus laevis retina.
1995,
Pubmed
,
Xenbase
Stiemke,
Outer segment disc membrane assembly in the absence of the pigment epithelium: the effect of exogenous sugars.
1994,
Pubmed
,
Xenbase
Travis,
The retinal degeneration slow (rds) gene product is a photoreceptor disc membrane-associated glycoprotein.
1991,
Pubmed
Usukura,
Changes in the localization and content of opsin during retinal development in the rds mutant mouse: immunocytochemistry and immunoassay.
1987,
Pubmed
Vardimon,
Expression of v-src in embryonic neural retina alters cell adhesion, inhibits histogenesis, and prevents induction of glutamine synthetase.
1991,
Pubmed
Witkovsky,
Photoreceptor thresholds and visual pigment levels in normal and vitamin A-deprived Xenopus tadpoles.
1976,
Pubmed
,
Xenbase
