ArticleInvestigative ophthalmology & visual science2012
CNGA3 deficiency affects cone synaptic terminal structure and function and leads to secondary rod dysfunction and degeneration.
Article in Investigative ophthalmology & visual science, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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19 citing papers in PubMed, 27 citations in OpenAlex.
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- Stimulation of Thyroid Hormone Signaling Induces Stress Responses in Mouse Retina.Advances in experimental medicine and biology · 2025Article
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- Deficiency of thyroid hormone receptor protects retinal pigment epithelium and photoreceptors from cell death in a mouse model of age-related macular degeneration.Cell death & disease · 2022Article
- Impact of Photoreceptor Loss on Retinal Circuitry.Annual review of vision science · 2021Review
- BBSome Component BBS5 Is Required for Cone Photoreceptor Protein Trafficking and Outer Segment Maintenance.Investigative ophthalmology & visual science · 2020Article
- Forward genetic analysis using OCT screening identifiesProceedings of the National Academy of Sciences of the United States of America · 2020Article
- Inhibition of thyroid hormone signaling protects retinal pigment epithelium and photoreceptors from cell death in a mouse model of age-related macular degeneration.Cell death & disease · 2020Article
- Cell-based therapeutic strategies for replacement and preservation in retinal degenerative diseases.Progress in retinal and eye research · 2017Review
- How to make a synaptic ribbon: RIBEYE deletion abolishes ribbons in retinal synapses and disrupts neurotransmitter release.The EMBO journal · 2016Article
- Activated mTORC1 promotes long-term cone survival in retinitis pigmentosa mice.The Journal of clinical investigation · 2015Article
- Homozygous missense variant in the human CNGA3 channel causes cone-rod dystrophy.European journal of human genetics : EJHG · 2015Article
- Autophagy supports color vision.Autophagy · 2015Article
- Loss of cone cyclic nucleotide-gated channel leads to alterations in light response modulating system and cellular stress response pathways: a gene expression profiling study.Human molecular genetics · 2013Article
- Establishing baseline rod electroretinogram values in achromatopsia and cone dystrophy.Documenta ophthalmologica. Advances in ophthalmology · 2012Article
- Bcl-xL-mediated remodeling of rod and cone synaptic mitochondria after postnatal lead exposure: electron microscopy, tomography and oxygen consumption.Molecular vision · 2012Article
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Authors and funding
7 authors at 6 institutions in 3 countries.
Funding
Abstract
purposeTo investigate rod function and survival after cone dysfunction and degeneration in a mouse model of cone cyclic nucleotide-gated (CNG) channel deficiency.
methodsRod function and survival in mice with cone CNG channel subunit CNGA3 deficiency (CNGA3-/- mice) were evaluated by electroretinographic (ERG), morphometric, and Western blot analyses. The arrangement, integrity, and ultrastructure of photoreceptor terminals were investigated by immunohistochemistry and electron microscopy.
resultsThe authors found loss of cone function and cone death accompanied by impairment of rods and rod-driven signaling in CNGA3-/- mice. Scotopic ERG b-wave amplitudes were reduced by 15% at 1 month, 30% at 6 months, and 40% at 9 months and older, while scotopic a-wave amplitudes were decreased by 20% at 9 months, compared with ERGs of age-matched wild-type mice. Outer nuclear layer thickness in CNGA3-/- retina was reduced by 15% at 12 months compared with age-matched wild-type controls. This was accompanied by a 30%-40% reduction in expression of rod-specific proteins, including rhodopsin, rod transducin α-subunit, and glutamic acid-rich protein (GARP). Cone terminals in the CNGA3-/- retina showed a progressive loss of neurochemical and ultrastructural integrity. Abnormalities were observed as early as 1 month. Disorganized rod terminal ultrastructure was noted by 12 months.
conclusionsThese findings demonstrate secondary rod impairment and degeneration after cone degeneration in mice with cone CNG channel deficiency. Loss of cone phototransduction accompanies the compromised integrity of cone terminals. With time, rod synaptic structure, function, and viability also become compromised.
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