{"doi":"10.17615/zyge-0571","title":"Mice Lacking Homer 1 Exhibit a Skeletal Myopathy Characterized by Abnormal Transient Receptor Potential Channel Activity","abstract":"Transient receptor potential (TRP) channels are nonselective cation channels, several of which are expressed in striated muscle. Because the scaffolding protein Homer 1 has been implicated in TRP channel regulation, we hypothesized that Homer proteins play a significant role in skeletal muscle function. Mice lacking Homer 1 exhibited a myopathy characterized by decreased muscle fiber cross-sectional area and decreased skeletal muscle force generation. Homer 1 knockout myotubes displayed increased basal current density and spontaneous cation influx. This spontaneous cation influx in Homer 1 knockout myotubes was blocked by reexpression of Homer 1b, but not Homer 1a, and by gene silencing of TRPC1. Moreover, diminished Homer 1 expression in mouse models of Duchenne's muscular dystrophy suggests that loss of Homer 1 scaffolding of TRP channels may contribute to the increased stretch-activated channel activity observed in mdx myofibers. These findings provide direct evidence that Homer 1 functions as an important scaffold for TRP channels and regulates mechanotransduction in skeletal muscle.","journal":"UNC Libraries","year":2020,"id":113510,"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":4,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9536,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2020-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":536022,"name":"J. Burch","orcid":null,"position":1,"is_corresponding":false},{"id":536023,"name":"R. S. Williams","orcid":null,"position":2,"is_corresponding":false},{"id":536024,"name":"P. F. Worley","orcid":null,"position":3,"is_corresponding":false},{"id":536025,"name":"Z.-S. Zhang","orcid":null,"position":4,"is_corresponding":false},{"id":536026,"name":"N. Yamaguchi","orcid":null,"position":5,"is_corresponding":false},{"id":522868,"name":"J. P. Eu","orcid":null,"position":6,"is_corresponding":false},{"id":320072,"name":"Paul B. Rosenberg","orcid":"0000-0003-1106-4299","position":7,"is_corresponding":false},{"id":536027,"name":"G. A. Truskey","orcid":null,"position":8,"is_corresponding":false},{"id":522870,"name":"G. Meissner","orcid":null,"position":9,"is_corresponding":false},{"id":536028,"name":"M. Seth","orcid":null,"position":10,"is_corresponding":false},{"id":536029,"name":"J. A. Stiber","orcid":null,"position":11,"is_corresponding":false},{"id":536030,"name":"R. Shah","orcid":null,"position":12,"is_corresponding":false},{"id":27181,"name":"S. Zhang","orcid":"0000-0002-8480-2662","position":0,"is_corresponding":true}],"reference_count":0,"raw_metadata":null,"created_at":"2026-07-18T23:13:21.458038Z","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":[]}