{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,11]],"date-time":"2025-11-11T05:43:49Z","timestamp":1762839829544,"version":"build-2065373602"},"reference-count":59,"publisher":"Wiley","issue":"24","license":[{"start":{"date-parts":[[2025,8,14]],"date-time":"2025-08-14T00:00:00Z","timestamp":1755129600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100010466","name":"Chancellerie des Universit\u00e9s de Paris","doi-asserted-by":"publisher","award":["DP 2018001655"],"award-info":[{"award-number":["DP 2018001655"]}],"id":[{"id":"10.13039\/501100010466","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001677","name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale","doi-asserted-by":"publisher","award":["RF20190502508"],"award-info":[{"award-number":["RF20190502508"]}],"id":[{"id":"10.13039\/501100001677","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100006342","name":"Association Vaincre la Mucoviscidose","doi-asserted-by":"publisher","award":["RF20200502716"],"award-info":[{"award-number":["RF20200502716"]}],"id":[{"id":"10.13039\/501100006342","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bpspubs.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["British J Pharmacology"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:sec>\n                    <jats:title>Background and Purpose<\/jats:title>\n                    <jats:p>\n                      Cystic fibrosis is an autosomal recessive disease caused by mutations in the\n                      <jats:italic>CFTR<\/jats:italic>\n                      gene, leading to progressive respiratory decline and reduced life expectancy. The most common mutation, CFTR\u2010F508del, results in mislocalised and non\u2010functional protein. Although triple therapy with elexacaftor\/tezacaftor\/ivacaftor (ETI) is prescribed for patients carrying this mutation, some biological defects remain unresolved. We previously identified COMMD1 as a potential therapeutic target, as its overexpression enhances CFTR\u2010WT plasma membrane localisation. CIGB\u2010552, a cell\u2010penetrating peptide discovered in 2013, stabilises COMMD1. This study evaluates its therapeutic potential in cystic fibrosis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Experimental Approach<\/jats:title>\n                    <jats:p>CIGB\u2010552 was tested, with and without ETI, in CFBE and HEK cells stably expressing CFTR\u2010WT or CFTR\u2010F508del, and in primary human bronchial cells. CFTR function was assessed using YFP quenching and short\u2010circuit current assays. Peptide uptake was evaluated using FITC\u2010labelled CIGB\u2010552 in submerged and air\u2013liquid interface models. Plasma membrane density of CFTR was measured in CFBE CFTR\u2010HA cells, and western blotting assessed CFTR maturation and COMMD1 expression.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Key results<\/jats:title>\n                    <jats:p>CIGB\u2010552 was non\u2010toxic and preferentially entered CFBE CFTR\u2010F508del cells rather than CFBE CFTR\u2010WT cells, without altering COMMD1 expression or localisation. Although not a corrector or potentiator alone, CIGB\u2010552 synergised with ETI, enhancing CFTR\u2010F508del\u2010mediated chloride efflux, confirmed in primary cells. CIGB\u2010552 also increased YFP quenching of CFTR\u2010WT and CFTR\u2010G551D, in combination with ivacaftor. This effect required COMMD1.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions and Implications<\/jats:title>\n                    <jats:p>COMMD1 expression was necessary for CIGB\u2010552 to affect CFTR function positively. Its synergy with the triple therapy offers a promising strategy for improving CF treatment.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/bph.70169","type":"journal-article","created":{"date-parts":[[2025,8,15]],"date-time":"2025-08-15T03:40:41Z","timestamp":1755229241000},"page":"5988-6006","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Peptide CIGB\u2010552 has a synergistic effect on CFTR\u2010F508del when combined with elexacaftor\/tezacaftor\/ivacaftor triple therapy for cystic fibrosis"],"prefix":"10.1111","volume":"182","author":[{"given":"Benjamin","family":"Simonneau","sequence":"first","affiliation":[{"name":"Univ Paris\u2010Est Creteil, INSERM, IMRB  Creteil France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"St\u00e9phanie","family":"Simon","sequence":"additional","affiliation":[{"name":"Univ Paris\u2010Est Creteil, INSERM, IMRB  Creteil 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