{"doi":"10.1161/circulationaha.123.064343","title":"Junctophilin-2 Regulates Mitochondrial Metabolism","abstract":"ight ventricular dysfunction (RVD) is a risk factor for death in multiple cardiovascular diseases, but approaches to combat RVD are lacking. 1Therapies used for left heart failure are largely ineffective in RVD, and thus the identification of molecules that augment right ventricular (RV) function could improve outcomes in a wide array of cardiac conditions.JPH2 (junctophilin-2) is an essential protein that has important roles in cardiomyocytes, including calcium handling and maintenance of t-tubule structure and gene transcription. 2In addition, JPH2 may regulate mitochondrial function, as Jph2 knockout mice exhibit cardiomyocyte mitochondrial swelling and cristae derangements. 3oreover, JPH2 knockdown in embryonic stem cellderived cardiomyocytes induces downregulation of the mitochondrial protein MFN2 (mitofusin-2), which disrupts mitochondrial cristae structure and transmembrane potential. 4Impaired mitochondrial metabolism drives RVD, 1 but the potential interaction of JPH2 with MFN2 and its effects on RV mitochondrial activity are not well-defined.Cellular fractionation, coimmunoprecipitation, and pulldown assays of recombinantly expressed and purified JPH2 and MFN2 constructs defined a direct JPH2-mitochondrial link.JPH2 was synthesized and cloned into pET151/dTOPO (Thermo) with deletions generated by Q5 mutagenesis polymerase chain reaction (NEB).His-tagged proteins were purified and tested for interaction by coimmunoprecipitation with MFN2 (Abcam), JPH2 (Invitrogen), isotype immunoglobulin G, and V5 (VWR) antibodies.Human induced pluripotent","journal":"Circulation","year":2024,"id":438903,"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":10,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9544,"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":937940,"name":"Lynn M. Hartweck","orcid":"0000-0002-7926-3342","position":1,"is_corresponding":false},{"id":292763,"name":"Felipe Kazmirczak","orcid":"0000-0001-8998-7259","position":2,"is_corresponding":false},{"id":1001192,"name":"Jenna B. Mendelson","orcid":"0000-0002-8229-385X","position":3,"is_corresponding":false},{"id":956069,"name":"Stephanie L. Deng","orcid":"0000-0002-0943-1339","position":4,"is_corresponding":false},{"id":851311,"name":"Madelyn Blake","orcid":"0000-0002-6802-9470","position":5,"is_corresponding":false},{"id":329252,"name":"Satadru K. Lahiri","orcid":"0000-0001-9502-1530","position":6,"is_corresponding":false},{"id":298615,"name":"Xander H.T. Wehrens","orcid":"0000-0001-5044-672X","position":7,"is_corresponding":false},{"id":394460,"name":"Kurt W. Prins","orcid":"0000-0002-0364-6742","position":8,"is_corresponding":false},{"id":394462,"name":"Sasha Z. Prisco","orcid":"0000-0002-9059-0635","position":0,"is_corresponding":true}],"reference_count":5,"raw_metadata":null,"created_at":"2026-07-19T02:00:43.618468Z","pmid":"39159221","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":[]}