{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,17]],"date-time":"2026-01-17T08:23:23Z","timestamp":1768638203233,"version":"3.49.0"},"reference-count":159,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2023,2,24]],"date-time":"2023-02-24T00:00:00Z","timestamp":1677196800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Cystic Fibrosis Foundation","award":["003055G221\u2013Gomes"],"award-info":[{"award-number":["003055G221\u2013Gomes"]}]},{"name":"Cystic Fibrosis Foundation","award":["CEECIND\/00048\/2017"],"award-info":[{"award-number":["CEECIND\/00048\/2017"]}]},{"name":"Individual Scientific Employment contract","award":["003055G221\u2013Gomes"],"award-info":[{"award-number":["003055G221\u2013Gomes"]}]},{"name":"Individual Scientific Employment contract","award":["CEECIND\/00048\/2017"],"award-info":[{"award-number":["CEECIND\/00048\/2017"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biomolecules"],"abstract":"<jats:p>Cystic fibrosis is a monogenic disease with a multisystemic phenotype, ranging from predisposition to chronic lung infection and inflammation to reduced bone mass. The exact mechanisms unbalancing the maintenance of an optimal bone mass in cystic fibrosis patients remain unknown. Multiple factors may contribute to severe bone mass reduction that, in turn, have devastating consequences in the patients\u2019 quality of life and longevity. Here, we will review the existing evidence linking the CFTR dysfunction and cell-intrinsic bone defects. Additionally, we will also address how the proinflammatory environment due to CFTR dysfunction in immune cells and chronic infection impairs the maintenance of an adequate bone mass in CF patients.<\/jats:p>","DOI":"10.3390\/biom13030425","type":"journal-article","created":{"date-parts":[[2023,2,24]],"date-time":"2023-02-24T02:32:12Z","timestamp":1677205932000},"page":"425","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6834-3224","authenticated-orcid":false,"given":"\u00d3scar","family":"Fonseca","sequence":"first","affiliation":[{"name":"i3S\u2013Instituto de Investiga\u00e7\u00e3o e Inova\u00e7\u00e3o em Sa\u00fade, Universidade do Porto, 4200-135 Porto, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7657-2118","authenticated-orcid":false,"given":"Maria Salom\u00e9","family":"Gomes","sequence":"additional","affiliation":[{"name":"i3S\u2013Instituto de Investiga\u00e7\u00e3o e Inova\u00e7\u00e3o em Sa\u00fade, Universidade do Porto, 4200-135 Porto, Portugal"},{"name":"ICBAS\u2013Instuto de Ci\u00eancias Biom\u00e9dicas de Abel Salazar, Universidade do Porto, 4030-313 Porto, Portugal"}]},{"given":"Maria Adelina","family":"Amorim","sequence":"additional","affiliation":[{"name":"CHUSJ\u2013Centro Hospital Universit\u00e1rio de S\u00e3o Jo\u00e3o, 4200-319 Porto, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5305-8538","authenticated-orcid":false,"given":"Ana Cordeiro","family":"Gomes","sequence":"additional","affiliation":[{"name":"i3S\u2013Instituto de Investiga\u00e7\u00e3o e Inova\u00e7\u00e3o em Sa\u00fade, Universidade do Porto, 4200-135 Porto, Portugal"},{"name":"IBMC\u2013Instituto de Biologia Molecular e Celular, Universidade do Porto, 4200-135 Porto, Portugal"}]}],"member":"1968","published-online":{"date-parts":[[2023,2,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2195","DOI":"10.1016\/S0140-6736(20)32542-3","article-title":"Cystic fibrosis","volume":"397","author":"Shteinberg","year":"2021","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1001\/archpedi.1938.01980140114013","article-title":"Cystic fibrosis of the pancreas and its relation to celiac disease: A clinical and pathologic study","volume":"56","author":"Andersen","year":"1938","journal-title":"Am. 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