Inner retina remodeling in a mouse model of stargardt-like macular dystrophy (STGD3)
| Autor: | Frederic Gaillard, Ian M. MacDonald, Silvina C. Mema, Paul R. Freund, Sharee Kuny, Janet R. Sparrow, Yves Sauve, Kang Zhang |
|---|---|
| Přispěvatelé: | Department of Physiology, University of Alberta, Institut de physiologie et biologie cellulaires (IPBC), Université de Poitiers-Centre National de la Recherche Scientifique (CNRS), Department of Ophthalmology, University of California [San Diego] (UC San Diego), University of California-University of California, Columbia University [New York] |
| Jazyk: | angličtina |
| Rok vydání: | 2010 |
| Předmět: |
Male
Aging Opsin Pathology genetic structures [SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology Retina homeostasis Pyridinium Compounds MESH: Electroretinography MESH: Genotype Macular Degeneration Mice chemistry.chemical_compound 0302 clinical medicine MESH: Eye Proteins MESH: Reverse Transcriptase Polymerase Chain Reaction MESH: Microscopy Confocal MESH: Aging MESH: Animals skin and connective tissue diseases Fluorescent Antibody Technique Indirect 0303 health sciences Microscopy Confocal medicine.diagnostic_test Reverse Transcriptase Polymerase Chain Reaction Articles Macular dystrophy Cell biology medicine.anatomical_structure MESH: Pyridinium Compounds Female MESH: Membrane Proteins Photoreceptor Cells Vertebrate Retinal Neurons cis-trans-Isomerases medicine.medical_specialty Genotype MESH: Mice Transgenic Mice Transgenic MESH: Carrier Proteins Biology Retinoids 03 medical and health sciences MESH: Mice Inbred C57BL Electroretinography medicine Animals MESH: Fluorescent Antibody Technique Indirect MESH: Retinoids Eye Proteins MESH: Macular Degeneration MESH: Mice MESH: Retinal Neurons 030304 developmental biology Retina MESH: Biological Markers Membrane Proteins Retinal Macular degeneration medicine.disease eye diseases MESH: Male Mice Inbred C57BL Disease Models Animal chemistry Cis-trans-Isomerases 030221 ophthalmology & optometry MESH: Photoreceptor Cells Vertebrate sense organs MESH: Disease Models Animal Carrier Proteins MESH: Female Biomarkers |
| Zdroj: | Investigative Ophthalmology & Visual Science Investigative Ophthalmology & Visual Science, Association for Research in Vision and Ophthalmology, 2010, 51 (4), pp.2248-62. ⟨10.1167/iovs.09-4718⟩ |
| ISSN: | 0146-0404 1552-5783 |
| Popis: | IF : 3,58); International audience; Purpose. To investigate the impact of progressive age-related photoreceptor degeneration on retinal integrity in Stargardt-like macular dystrophy (STGD3). Methods. The structural design of the inner retina of the ELOVL4 transgenic mouse model of STGD3 was compared with that of age-matched littermate wild-type (WT) mice from 1 to 24 months of age by using immunohistofluorescence and confocal microscopy and by relying on antibodies against cell-type-specific markers, synapse-associated proteins, and neurotransmitters. Results. Müller cell reactivity occurred at the earliest age studied, before photoreceptor loss. This finding is perhaps not surprising, considering the cell's ubiquitous roles in retina homeostasis. Second-order neurons displayed salient morphologic changes as a function of photoreceptoral input loss. Age-related sprouting of dendritic fibers from rod bipolar and horizontal cells into the ONL did not occur. In contrast, with the loss of photoreceptor sensory input, these second-order neurons progressively bore fewer synapses. After rod loss, the few remaining cones showed abnormal opsin expression, revealing tortuous branched axons. After complete ONL loss (beyond 18 months of age), localized areas of extreme retinal disruptions were observed in the central retina. RPE cell invasion, dense networks of strongly reactive Müller cell processes, and invagination of axons and blood vessels were distinctive features of these regions. In addition, otherwise unaffected cholinergic amacrine cells displayed severe perturbation of their cell bodies and synaptic plexi in these areas. Conclusions. Remodeling in ELOVL4 transgenic mice follows a pattern similar to that reported after other types of hereditary retinopathies in animals and humans, pointing to a potentially common pathophysiologic mechanism. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|