{"doi":"10.1167/iovs.10-6358","title":"Early-Onset, Slow Progression of Cone Photoreceptor Dysfunction and Degeneration in CNG Channel Subunit CNGB3 Deficiency","abstract":null,"journal":"Investigative Opthalmology & Visual Science","year":2011,"id":650267,"datarank":0.5641800173540344,"base_score":3.7612001156935624,"endowment":3.7612001156935624,"self_citation_contribution":0.5641800173540344,"citation_network_contribution":0.0,"self_endowment_contribution":0.5641800173540344,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":42,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":240659,"name":"Lynsie Morris","orcid":"0000-0003-0701-271X","position":1,"is_corresponding":false},{"id":415861,"name":"Steven J. Fliesler","orcid":"0000-0002-2557-142X","position":2,"is_corresponding":false},{"id":363383,"name":"David M. Sherry","orcid":"0000-0003-1568-0611","position":3,"is_corresponding":false},{"id":1302030,"name":"Xi-Qin Ding","orcid":null,"position":4,"is_corresponding":false},{"id":438070,"name":"Jianhua Xu","orcid":"0000-0001-7585-3014","position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Early-Onset, Slow Progression of Cone Photoreceptor Dysfunction and Degeneration in CNG Channel Subunit CNGB3 Deficiency","abstract":"PURPOSE: To investigate the progression of cone dysfunction and degeneration in CNG channel subunit CNGB3 deficiency. METHODS: Retinal structure and function in CNGB3(-/-) and wild-type (WT) mice were evaluated by electroretinography (ERG), lectin cytochemistry, and correlative Western blot analysis of cone-specific proteins. Cone and rod terminal integrity was assessed by electron microscopy and synaptic protein immunohistochemical distribution. RESULTS: Cone ERG amplitudes (photopic b-wave) in CNGB3(-/-) mice were reduced to approximately 50% of WT levels by postnatal day 15, decreasing further to approximately 30% of WT levels by 1 month and to approximately 20% by 12 months of age. Rod ERG responses (scotopic a-wave) were not affected in CNGB3(-/-) mice. Average CNGB3(-/-) cone densities were approximately 80% of WT levels at 1 month and declined slowly thereafter to only approximately 50% of WT levels by 12 months. Expression levels of M-opsin, cone transducin α-subunit, and cone arrestin in CNGB3(-/-) mice were reduced by 50% to 60% by 1 month and declined to 35% to 45% of WT levels by 9 months. In addition, cone opsin mislocalized to the outer nuclear layer and the outer plexiform layer in the CNGB3(-/-) retina. Cone and rod synaptic marker expression and terminal ultrastructure were normal in the CNGB3(-/-) retina. CONCLUSIONS: These findings are consistent with an early-onset, slow progression of cone functional defects and cone loss in CNGB3(-/-) mice, with the cone signaling deficits arising from disrupted phototransduction and cone loss rather than from synaptic defects.","is_dataset_classified":null,"base_score":3.7612001156935624,"endowment":3.7612001156935624,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"21273547","pmcid":"PMC3109041","openalex_id":"https://openalex.org/W2000328471","authors":[],"funders":[{"funder_name":"NCRR NIH HHS","grant_id":"P20 RR017703","title":null},{"funder_name":"NEI NIH HHS","grant_id":"P30 EY12190","title":null},{"funder_name":"NEI NIH HHS","grant_id":"P30 EY012190","title":null},{"funder_name":"NEI NIH HHS","grant_id":"R01 EY007361","title":null},{"funder_name":"NEI NIH HHS","grant_id":"R01 EY019490","title":null}],"total_grants":5,"fwci":1.582,"citation_percentile":0.82017774,"influential_citations":0,"citation_trend":[{"year":2012,"count":3},{"year":2013,"count":5},{"year":2014,"count":2},{"year":2015,"count":6},{"year":2016,"count":3},{"year":2017,"count":3},{"year":2018,"count":3},{"year":2019,"count":1},{"year":2020,"count":3},{"year":2021,"count":4},{"year":2023,"count":3},{"year":2024,"count":1},{"year":2025,"count":2}],"oa_status":"bronze","license":null,"oa_locations":[{"url":"https://iovs.arvojournals.org/arvo/content_public/journal/iovs/933250/z7g00611003557.pdf","host_type":"journal"},{"url":"https://iovs.arvojournals.org/arvo/content_public/journal/iovs/933250/z7g00611003557.pdf","host_type":"publisher"},{"url":"http://iovs.arvojournals.org/article.aspx?doi=10.1167/iovs.10-6358","host_type":"publisher"},{"url":"https://doi.org/10.1167/iovs.10-6358","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/21273547","host_type":"repository"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/3109041","host_type":"repository"}],"fields_of_study":["Retinal Development and Disorders","Glaucoma and retinal disorders","Cellular transport and secretion"],"mesh_terms":["Animals","Electrophoresis, Polyacrylamide Gel","Electroretinography","Mice, Inbred C57BL","Microscopy, Electron","Retinal Degeneration","Blotting, Western","Transducin","Retinal Cone Photoreceptor Cells","Mice, Knockout","Microscopy, Confocal","Fluorescent Antibody Technique, Indirect","Arrestin","Mice","Cyclic Nucleotide-Gated Cation Channels","Opsins"],"keywords":["Transducin","Erg","Electroretinography","Visual phototransduction","Achromatopsia","Opsin","Outer nuclear layer","Scotopic vision","Photopic vision","Biology","Retinal degeneration","Retina","Retinitis pigmentosa","Retinal","Cell biology","Biophysics","Molecular biology","Rhodopsin","Neuroscience","Biochemistry"],"sdg_mappings":[],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-10T05:10:36.910385Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}