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CRB2 Loss in Rod Photoreceptors Is Associated with Progressive Loss of Retinal Contrast Sensitivity
- Source :
- International Journal of Molecular Sciences, Volume 20, Issue 17, International Journal of Molecular Sciences, 20(17). Multidisciplinary Digital Publishing Institute (MDPI), International Journal of Molecular Sciences, 20(17). MDPI, International Journal of Molecular Sciences, Vol 20, Iss 17, p 4069 (2019)
- Publication Year :
- 2019
- Publisher :
- Multidisciplinary Digital Publishing Institute, 2019.
-
Abstract
- Variations in the Crumbs homolog-1 (CRB1) gene are associated with a wide variety of autosomal recessive retinal dystrophies, including early onset retinitis pigmentosa (RP) and Leber congenital amaurosis (LCA). CRB1 belongs to the Crumbs family, which in mammals includes CRB2 and CRB3. Here, we studied the specific roles of CRB2 in rod photoreceptor cells and whether ablation of CRB2 in rods exacerbates the Crb1-disease. Therefore, we assessed the morphological, retinal, and visual functional consequences of specific ablation of CRB2 from rods with or without concomitant loss of CRB1. Our data demonstrated that loss of CRB2 in mature rods resulted in RP. The retina showed gliosis and disruption of the subapical region and adherens junctions at the outer limiting membrane. Rods were lost at the peripheral and central superior retina, while gross retinal lamination was preserved. Rod function as measured by electroretinography was impaired in adult mice. Additional loss of CRB1 exacerbated the retinal phenotype leading to an early reduction of the dark-adapted rod photoreceptor a-wave and reduced contrast sensitivity from 3-months-of-age, as measured by optokinetic tracking reflex (OKT) behavior testing. The data suggest that CRB2 present in rods is required to prevent photoreceptor degeneration and vision loss.
- Subjects :
- 0301 basic medicine
Retinal degeneration
retina
genetic structures
030105 genetics & heredity
lcsh:Chemistry
Mice
chemistry.chemical_compound
Retinal Rod Photoreceptor Cells
lcsh:QH301-705.5
Spectroscopy
Mice, Knockout
CRB1
medicine.diagnostic_test
photoreceptors
General Medicine
Immunohistochemistry
crumbs homolog-1
Computer Science Applications
Cell biology
medicine.anatomical_structure
Retinal Dystrophies
Biology
Leber congenital amaurosis
Article
Catalysis
Contrast Sensitivity
Inorganic Chemistry
03 medical and health sciences
rod photoreceptors
Microscopy, Electron, Transmission
retinitis pigmentosa
Retinitis pigmentosa
Electroretinography
medicine
Animals
Outer Limiting Membrane
crumbs complex
Physical and Theoretical Chemistry
Molecular Biology
Retina
Organic Chemistry
Membrane Proteins
Retinal
medicine.disease
eye diseases
Mice, Inbred C57BL
Disease Models, Animal
030104 developmental biology
lcsh:Biology (General)
lcsh:QD1-999
chemistry
retinal degeneration
sense organs
Subjects
Details
- Language :
- English
- ISSN :
- 14220067 and 16616596
- Database :
- OpenAIRE
- Journal :
- International Journal of Molecular Sciences
- Accession number :
- edsair.doi.dedup.....1c8ee38415c7a73ffcb5e82627ad0bbb
- Full Text :
- https://doi.org/10.3390/ijms20174069