{"doi":"10.17615/0mcx-w663","title":"Salt restriction induces pseudohypoaldosteronism type 1 in mice expressing low levels of the  -subunit of the amiloride-sensitive epithelial sodium channel","abstract":"The amiloride-sensitive epithelial sodium channel (ENaC) is a heteromultimer of three homologous subunits (α-, β-, and γ-subunits). To study the role of the β-subunit in vivo, we analyzed mice in which the βENaC gene locus was disrupted. These mice showed low levels of βENaC mRNA expression in kidney (≈1%), lung (≈1%), and colon (≈4%). In homozygous mutant βENaC mice, no βENaC protein could be detected with immunofluorescent staining. At birth, there was a small delay in lung-liquid clearance that paralleled diminished amiloride-sensitive Na+ absorption in tracheal explants. With normal salt intake, these mice showed a normal growth rate. However, in vivo, adult βENaC m/m mice exhibited a significantly reduced ENaC activity in colon and elevated plasma aldosterone levels, suggesting hypovolemia and pseudohypoaldosteronism type 1. This phenotype was clinically silent, as βENaC m/m mice showed no weight loss, normal plasma Na+ and K+ concentrations, normal blood pressure, and a compensated metabolic acidosis. On low-salt diets, βENaC-mutant mice developed clinical symptoms of an acute pseudohypoaldosteronism type 1 (weight loss, hyperkalemia, and decreased blood pressure), indicating that βENaC is required for Na+ conservation during salt deprivation.","journal":"UNC Libraries","year":2020,"id":136975,"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":0,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9548,"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":72858,"name":"Q. Wang","orcid":null,"position":1,"is_corresponding":false},{"id":540094,"name":"M. Burnier","orcid":null,"position":2,"is_corresponding":false},{"id":596823,"name":"S. A. Ernst","orcid":null,"position":3,"is_corresponding":false},{"id":412655,"name":"J. T. Gatzy","orcid":null,"position":4,"is_corresponding":false},{"id":596824,"name":"S. Pradervand","orcid":null,"position":5,"is_corresponding":false},{"id":412653,"name":"B. R. Grubb","orcid":null,"position":6,"is_corresponding":false},{"id":46280,"name":"A. Schmidt","orcid":"0000-0003-2711-8984","position":7,"is_corresponding":false},{"id":540090,"name":"E. Hummler","orcid":null,"position":8,"is_corresponding":false},{"id":540095,"name":"B. C. Rossier","orcid":null,"position":9,"is_corresponding":false},{"id":596825,"name":"P. M. Barker","orcid":null,"position":10,"is_corresponding":false},{"id":596826,"name":"R. J. M. Bindels","orcid":null,"position":11,"is_corresponding":false},{"id":540097,"name":"F. Beermann","orcid":null,"position":0,"is_corresponding":true}],"reference_count":0,"raw_metadata":null,"created_at":"2026-07-18T23:16:52.487376Z","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":[]}