{"doi":"10.1084/jem.20120562","title":"Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype","abstract":"<jats:p>Cystic fibrosis (CF) is caused by a nonfunctional chloride and bicarbonate ion channel (CF transmembrane regulator [CFTR]), but the link to the phenomenon of stagnant mucus is not well understood. Mice lacking functional CFTR (CftrΔ508) have no lung phenotype but show similar ileal problems to humans. We show that the ileal mucosa in CF have a mucus that adhered to the epithelium, was denser, and was less penetrable than that of wild-type mice. The properties of the ileal mucus of CF mice were normalized by secretion into a high concentration sodium bicarbonate buffer (∼100 mM). In addition, bicarbonate added to already formed CF mucus almost completely restored the mucus properties. This knowledge may provide novel therapeutic options for CF.</jats:p>","journal":"Journal of Experimental Medicine","year":2012,"id":607437,"datarank":0.8765316625547042,"base_score":5.84354441703136,"endowment":5.84354441703136,"self_citation_contribution":0.8765316625547042,"citation_network_contribution":0.0,"self_endowment_contribution":0.8765316625547042,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":344,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":304121,"name":"Anna Ermund","orcid":"0000-0002-3233-043X","position":1,"is_corresponding":false},{"id":1559762,"name":"Daniel Ambort","orcid":null,"position":2,"is_corresponding":false},{"id":1559763,"name":"Malin E.V. 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In addition, bicarbonate added to already formed CF mucus almost completely restored the mucus properties. This knowledge may provide novel therapeutic options for CF.</jats:p>","is_dataset_classified":null,"base_score":5.84354441703136,"endowment":5.84354441703136,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"22711878","pmcid":"PMC3405509","openalex_id":"https://openalex.org/W2113830910","authors":[],"funders":[{"funder_name":"European Commission FP7","grant_id":"FP7_200931","title":null},{"funder_name":"NIAID NIH HHS","grant_id":"U01 AI095473","title":null},{"funder_name":"European Commission","grant_id":"200931","title":"Mucosal protease and their inhibitors in inflammatory bowel disease: From etiopathogenetic insight to innovative therapy"}],"total_grants":3,"fwci":21.8444,"citation_percentile":0.99661711,"influential_citations":0,"citation_trend":[{"year":2012,"count":10},{"year":2013,"count":24},{"year":2014,"count":31},{"year":2015,"count":35},{"year":2016,"count":26},{"year":2017,"count":25},{"year":2018,"count":25},{"year":2019,"count":29},{"year":2020,"count":20},{"year":2021,"count":24},{"year":2022,"count":28},{"year":2023,"count":24},{"year":2024,"count":18},{"year":2025,"count":15},{"year":2026,"count":10}],"oa_status":"bronze","license":"CC BY NC SA","oa_locations":[{"url":"http://jem.rupress.org/content/jem/209/7/1263.full.pdf","host_type":"journal"},{"url":"http://jem.rupress.org/content/jem/209/7/1263.full.pdf","host_type":"publisher"},{"url":"https://rupress.org/jem/article-pdf/209/7/1263/1753443/jem_20120562.pdf","host_type":"publisher"},{"url":"https://doi.org/10.1084/jem.20120562","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/22711878","host_type":"repository"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/3405509","host_type":"repository"},{"url":"https://research.chalmers.se/en/publication/169044","host_type":"repository"},{"url":"https://europepmc.org/articles/PMC3405509","host_type":"Europe_PMC"},{"url":"https://europepmc.org/articles/PMC3405509?pdf=render","host_type":"Europe_PMC"},{"url":"http://dx.doi.org/10.1084/jem.20120562","host_type":""},{"url":"https://dx.doi.org/10.1084/jem.20120562","host_type":""},{"url":"http://urn.kb.se/resolve?urn=urn:nbn:se:kth:diva-160351","host_type":""}],"fields_of_study":["Cystic Fibrosis Research Advances","Neonatal Respiratory Health Research","Tracheal and airway disorders","0301 basic medicine","03 medical and health sciences","0303 health sciences","Animals","Bicarbonates","Cystic Fibrosis","Cystic Fibrosis Transmembrane Conductance Regulator","Dose-Response Relationship, Drug","Epithelium","Female","Ileum","Immunohistochemistry","In Vitro Techniques","Intestinal Mucosa","Intestine, Small","Male","Mice","Mice, Inbred C57BL","Mice, Knockout","Microscopy, Confocal","Microscopy, Electron, Transmission","Mucins","Mucus","Phenotype"],"mesh_terms":["Animals","Bicarbonates","Cystic Fibrosis","Dose-Response Relationship, Drug","Epithelium","Female","Ileum","Immunohistochemistry","Intestinal Mucosa","Intestine, Small","Male","Mice, Inbred C57BL","Mucins","Mucus","Phenotype","Mice, Knockout","Microscopy, Confocal","Cystic Fibrosis Transmembrane Conductance Regulator","Microscopy, Electron, Transmission","Mice","In Vitro Techniques"],"keywords":["Cystic fibrosis","Mucus","Mucin","Bicarbonate","Cystic fibrosis transmembrane conductance regulator","Secretion","Epithelium","Chemistry","Phenotype","Biology","Mucin 2","Chloride channel","Transepithelial potential difference","Cell biology","Internal medicine","Endocrinology","Biochemistry","Ion transporter","Medicine","Membrane","Gene expression","Male","Cell- och molekylärbiologi","In Vitro Techniques","Article","Mice","Microscopy, Electron, Transmission","Ileum","Intestine, Small","Animals","Intestinal Mucosa","Mice, Knockout","Microscopy, Confocal","Dose-Response Relationship, Drug","Mucins","Immunohistochemistry","Mice, Inbred C57BL","Bicarbonates","Female","Cell and Molecular Biology"],"sdg_mappings":[{"sdg_number":3,"sdg_label":"3. 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