{"doi":"10.1038/ncomms1101","title":"A β-synuclein mutation linked to dementia produces neurodegeneration when expressed in mouse brain","abstract":null,"journal":"Nature Communications","year":2010,"id":641924,"datarank":0.6515708132780527,"base_score":4.343805421853684,"endowment":4.343805421853684,"self_citation_contribution":0.6515708132780527,"citation_network_contribution":0.0,"self_endowment_contribution":0.6515708132780527,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":76,"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":1669306,"name":"Shuei Sugama","orcid":null,"position":1,"is_corresponding":false},{"id":1669307,"name":"Kazunari Sekiyama","orcid":null,"position":2,"is_corresponding":false},{"id":1669308,"name":"Akio Sekigawa","orcid":null,"position":3,"is_corresponding":false},{"id":1669309,"name":"Tohru Tsukui","orcid":null,"position":4,"is_corresponding":false},{"id":1669310,"name":"Masaaki Nakai","orcid":null,"position":5,"is_corresponding":false},{"id":272191,"name":"Masaaki Waragai","orcid":"0000-0003-4874-6154","position":6,"is_corresponding":false},{"id":1524917,"name":"Takato Takenouchi","orcid":null,"position":7,"is_corresponding":false},{"id":1669311,"name":"Yoshiki Takamatsu","orcid":null,"position":8,"is_corresponding":false},{"id":1505111,"name":"Jianshe Wei","orcid":null,"position":9,"is_corresponding":false},{"id":480540,"name":"Edward Rockenstein","orcid":"0000-0001-8905-2859","position":10,"is_corresponding":false},{"id":1669312,"name":"Albert R. 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In this paper, we show that transgenic (tg) mice expressing DLB-linked P123H βS develop progressive neurodegeneration, as characterized by axonal swelling, astrogliosis and behavioural abnormalities, with memory disorder being more prominent than motor deficits. Furthermore, cross-breeding of P123H βS tg mice with αS tg mice, but not with αS knockout mice, greatly enhanced neurodegeneration phenotypes. These results suggest that P123H βS is pathogenic and cooperates with pathogenic αS to stimulate neurodegeneration in mouse brain, indicating a causative role of P123H βS in familial DLB. Given the neuritic pathology of βS in sporadic α-synucleinopathies, it appears that alteration of βS can contribute to the pathogenesis of a broad range of α-synucleinopathies. Little is known about β-synuclein mutations in neurological disease. In this article, the authors demonstrate that mice with a mutation in β-synuclein show progressive neurodegenerative disease and suggest that this mutation can enhance the brain defects caused by α-synuclein mutations in mice.","is_dataset_classified":null,"base_score":4.343805421853684,"endowment":4.343805421853684,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"21045828","pmcid":"PMC3060620","openalex_id":"https://openalex.org/W1988094412","authors":[],"funders":[{"funder_name":"NIA NIH HHS","grant_id":"P01 AG010435","title":null},{"funder_name":"NIA NIH HHS","grant_id":"P01 AG022074","title":null},{"funder_name":"NIEHS NIH HHS","grant_id":"P01 ES016731","title":null},{"funder_name":"NIA NIH HHS","grant_id":"R37 AG018440","title":null},{"funder_name":"NIA NIH HHS","grant_id":"R01 AG018440","title":null}],"total_grants":5,"fwci":4.4026,"citation_percentile":0.94442958,"influential_citations":0,"citation_trend":[{"year":2012,"count":9},{"year":2013,"count":9},{"year":2014,"count":8},{"year":2015,"count":7},{"year":2016,"count":5},{"year":2017,"count":4},{"year":2018,"count":11},{"year":2019,"count":2},{"year":2020,"count":3},{"year":2021,"count":3},{"year":2022,"count":2},{"year":2023,"count":2},{"year":2024,"count":1},{"year":2025,"count":2},{"year":2026,"count":1}],"oa_status":"hybrid","license":"cc-by-nc-sa","oa_locations":[{"url":"https://www.nature.com/articles/ncomms1101.pdf","host_type":"journal"},{"url":"https://www.nature.com/articles/ncomms1101.pdf","host_type":"publisher"},{"url":"https://www.nature.com/articles/ncomms1101","host_type":"publisher"},{"url":"https://doi.org/10.1038/ncomms1101","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/21045828","host_type":"repository"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/3060620","host_type":"repository"},{"url":"https://europepmc.org/articles/PMC3060620","host_type":"Europe_PMC"},{"url":"https://europepmc.org/articles/PMC3060620?pdf=render","host_type":"Europe_PMC"}],"fields_of_study":["Parkinson's Disease Mechanisms and Treatments","Nuclear Receptors and Signaling","Neurological diseases and metabolism"],"mesh_terms":[],"keywords":["Neurodegeneration","Synucleinopathies","Dementia with Lewy bodies","Pathogenesis","Synuclein","Neuroscience","Astrogliosis","Biology","Parkinson's disease","Alpha-synuclein","Dementia","Genetically modified mouse","Phenotype","Transgene","Pathology","Medicine","Disease","Genetics","Immunology","Gene","Central nervous system"],"sdg_mappings":[{"sdg_number":0,"sdg_label":"Good health and well-being"}],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-07T20:55:44.386034Z","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":[]}