{"doi":"10.1126/science.1134108","title":"Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis","abstract":"Ubiquitin-positive, tau- and alpha-synuclein-negative inclusions are hallmarks of frontotemporal lobar degeneration with ubiquitin-positive inclusions and amyotrophic lateral sclerosis. Although the identity of the ubiquitinated protein specific to either disorder was unknown, we showed that TDP-43 is the major disease protein in both disorders. Pathologic TDP-43 was hyper-phosphorylated, ubiquitinated, and cleaved to generate C-terminal fragments and was recovered only from affected central nervous system regions, including hippocampus, neocortex, and spinal cord. TDP-43 represents the common pathologic substrate linking these neurodegenerative disorders.","journal":"Science","year":2006,"id":6296,"datarank":14.114974721119193,"base_score":8.787067645218054,"endowment":8.787067645218054,"self_citation_contribution":1.3180601467827082,"citation_network_contribution":12.796914574336485,"self_endowment_contribution":1.3180601467827082,"citer_contribution":12.796914574336485,"corpus_percentile":87.4,"corpus_rank":1916,"citation_count":6548,"citer_count":159,"citers_with_citation_signal":159,"citers_with_endowment":159,"datacite_reuse_total":0,"is_dataset":false,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2006-10-06","authors":[{"id":58503,"name":"Deepak M. Sampathu","orcid":null,"position":1,"is_corresponding":false},{"id":58504,"name":"Linda K. Kwong","orcid":null,"position":2,"is_corresponding":false},{"id":58505,"name":"Adam C. Truax","orcid":null,"position":3,"is_corresponding":false},{"id":58506,"name":"Matthew C. Micsenyi","orcid":null,"position":4,"is_corresponding":false},{"id":58507,"name":"Thomas T. Chou","orcid":null,"position":5,"is_corresponding":false},{"id":58508,"name":"Jennifer Bruce","orcid":"0000-0001-7830-5937","position":6,"is_corresponding":false},{"id":58509,"name":"Theresa Schuck","orcid":null,"position":7,"is_corresponding":false},{"id":58510,"name":"Murray Grossman","orcid":"0000-0002-7447-6218","position":8,"is_corresponding":false},{"id":58511,"name":"Christopher M. Clark","orcid":"0000-0002-4716-4155","position":9,"is_corresponding":false},{"id":58512,"name":"Leo F. McCluskey","orcid":null,"position":10,"is_corresponding":false},{"id":58513,"name":"Bruce L. Miller","orcid":"0000-0002-2152-4220","position":11,"is_corresponding":false},{"id":58514,"name":"Eliezer Masliah","orcid":"0000-0002-2117-5569","position":12,"is_corresponding":false},{"id":58515,"name":"Ian R. Mackenzie","orcid":"0000-0003-3875-2972","position":13,"is_corresponding":false},{"id":58516,"name":"Howard Feldman","orcid":"0000-0002-9258-4538","position":14,"is_corresponding":false},{"id":58517,"name":"Wolfgang Feiden","orcid":null,"position":15,"is_corresponding":false},{"id":58518,"name":"Hans A. Kretzschmar","orcid":null,"position":16,"is_corresponding":false},{"id":27606,"name":"John Q. Trojanowski","orcid":"0000-0002-9239-8794","position":17,"is_corresponding":false},{"id":58520,"name":"Leo McCluskey","orcid":null,"position":19,"is_corresponding":false},{"id":58521,"name":"W. Feiden","orcid":null,"position":20,"is_corresponding":false},{"id":27604,"name":"Virginia M.‐Y. Lee","orcid":"0000-0003-3536-6902","position":21,"is_corresponding":false},{"id":58502,"name":"Manuela Neumann","orcid":"0000-0002-6232-4057","position":0,"is_corresponding":true}],"reference_count":29,"raw_metadata":{"citation_network_status":"fetched"},"created_at":"2026-03-01T18:20:47.508186Z","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,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}