{"doi":"10.1101/2024.07.18.604174","title":"Amyloid fibril structures link CHCHD10 and CHCHD2 to neurodegeneration","abstract":"CHCHD10 is mutated in rare cases of FTD and ALS and aggregates in mouse models of disease. Here we show that the disordered N-terminal domain of CHCHD10 forms amyloid fibrils and report their cryoEM structure. Disease-associated mutations cannot be accommodated by the WT fibril structure, while sequence differences between CHCHD10 and CHCHD2 are tolerated, explaining the co-aggregation of the two proteins and linking CHCHD10 and CHCHD2 amyloid fibrils to neurodegeneration.","journal":"bioRxiv (Cold Spring Harbor Laboratory)","year":2024,"id":491455,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":1,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.947,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2024-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":883819,"name":"Nicole M. Sayles","orcid":"0000-0002-7653-4282","position":1,"is_corresponding":false},{"id":317546,"name":"Yun Huang","orcid":"0000-0002-2991-6122","position":2,"is_corresponding":false},{"id":686872,"name":"Chiara Mancinelli","orcid":"0000-0002-2653-4698","position":3,"is_corresponding":false},{"id":348836,"name":"Kevin McAvoy","orcid":"0000-0002-0025-1921","position":4,"is_corresponding":false},{"id":550624,"name":"Neil A. Shneider","orcid":"0000-0002-3223-7366","position":5,"is_corresponding":false},{"id":368949,"name":"Giovanni Manfredi","orcid":"0000-0003-3893-1348","position":6,"is_corresponding":false},{"id":383951,"name":"Hibiki Kawamata","orcid":"0000-0003-0020-6933","position":7,"is_corresponding":false},{"id":317552,"name":"David Eliezer","orcid":"0000-0002-1311-7537","position":8,"is_corresponding":false},{"id":317547,"name":"Guohua Lv","orcid":"0000-0002-5076-096X","position":0,"is_corresponding":true}],"reference_count":25,"raw_metadata":null,"created_at":"2026-07-19T02:08:45.247225Z","pmid":"39091724","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":[]}