Spatio-temporal characterization of S- and M/L-cone degeneration in the Rd1 mouse model of retinitis pigmentosa
| Autor: | Jack Ao, Glyn Chidlow, Robert J Casson, John P. M. Wood, Daniel S. Narayan |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2019 |
| Předmět: |
Time Factors
genetic structures Cell Count Degeneration (medical) Rd1 mouse Mice chemistry.chemical_compound 0302 clinical medicine Dual cone and polar cone Contrast (vision) media_common General Neuroscience Retinal Degeneration lcsh:QP351-495 Anatomy Retinitis pigmentosa medicine.anatomical_structure Retinal Cone Photoreceptor Cells Microglia Dual cone Research Article M/L-opsin media_common.quotation_subject Biology Retina lcsh:RC321-571 03 medical and health sciences Cellular and Molecular Neuroscience medicine Animals lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry Cyclic Nucleotide Phosphodiesterases Type 6 Opsins S-opsin Retinal Cone (category theory) Cone photoreceptor Retinal Photoreceptor Cell Outer Segment medicine.disease Mice Mutant Strains Cone cell eye diseases Disease Models Animal lcsh:Neurophysiology and neuropsychology chemistry Nerve Degeneration Degeneration Outer segment sense organs 030217 neurology & neurosurgery |
| Zdroj: | BMC Neuroscience, Vol 20, Iss 1, Pp 1-17 (2019) BMC Neuroscience |
| ISSN: | 1471-2202 |
| DOI: | 10.1186/s12868-019-0528-2 |
| Popis: | Background The Pde6brd1 (Rd1) mouse is widely used as a murine model for human retinitis pigmentosa. Understanding the spatio-temporal patterns of cone degeneration is important for evaluating potential treatments. In the present study we performed a systematic characterization of the spatio-temporal patterns of S- and M/L-opsin + cone outer segment and cell body degeneration in Rd1 mice, described the distribution and proportion of dual cones in Rd1 retinas, and examined the kinetics of microglial activation during the period of cone degeneration. Results Outer segments of S- and M/L-cones degenerated far more rapidly than their somas. Loss of both S- and M/L-opsin + outer segments was fundamentally complete by P21 in the central retina, and 90% complete by P45 in the peripheral retina. In comparison, degeneration of S- and M/L-opsin + cell bodies proceeded at a slower rate. There was a marked hemispheric asymmetry in the rate of S-opsin + and M/L-opsin + cell body degeneration. M/L-opsin + cones were more resilient to degeneration in the superior retina, whilst S-opsin + cones were relatively preserved in the inferior retina. In addition, cone outer segment and cell body degeneration occurred far more rapidly in the central than the peripheral retina. At P14, the superior retina comprised a minority of genuine S-cones with a much greater complement of genuine M/L-opsin cones and dual cones, whilst the other three retinal quadrants had broadly similar numbers of genuine S-cones, genuine M/L-cones and dual cones. At P60, approximately 50% of surviving cones in the superior, nasal and temporal quadrants were dual cones. In contrast, the inferior peripheral retina at P60 contained almost exclusively genuine S-cones with a tiny minority of dual cones. Microglial number and activity were stimulated during rod breakdown, remained relatively high during cone outer segment degeneration and loss of cone somas in the central retina, and decreased thereafter in the period coincident with slow degeneration of cone cell bodies in the peripheral retina. Conclusion The results of the present study provide valuable insights into cone degeneration in the Rd1 mouse, substantiating and extending conclusions drawn from earlier studies. |
| Databáze: | OpenAIRE |
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