{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T22:16:55Z","timestamp":1791584215356,"version":"4.3.4"},"reference-count":39,"publisher":"European Respiratory Society (ERS)","issue":"5","content-domain":{"domain":["ersjournals.com"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2014,7,14]]},"published-print":{"date-parts":[[2014,11]]},"abstract":"<jats:p>We have previously reported cyanide at concentrations of up to 150 \u03bcM in the sputum of cystic fibrosis patients infected with<jats:italic>Pseudomonas aeruginosa<\/jats:italic>and a negative correlation with lung function. Our aim was to investigate possible mechanisms for this association, focusing on the effect of pathophysiologically relevant cyanide levels on human respiratory cell function.<\/jats:p><jats:p>Ciliary beat frequency measurements were performed on nasal brushings and nasal air\u2013liquid interface (ALI) cultures obtained from healthy volunteers and cystic fibrosis patients.<\/jats:p><jats:p>Potassium cyanide decreased ciliary beat frequency in healthy nasal brushings (n = 6) after 60 min (150 \u03bcM: 47% fall, p&lt;0.0012; 75 \u03bcM: 32% fall, p&lt;0.0001). Samples from cystic fibrosis patients (n = 3) showed similar results (150 \u03bcM: 55% fall, p = 0.001). Ciliary beat frequency inhibition was not due to loss of cell viability and was reversible. The inhibitory mechanism was independent of ATP levels. KCN also significantly inhibited ciliary beat frequency in ALI cultures, albeit to a lesser extent. Ciliary beat frequency measurements on ALI cultures treated with culture supernatants from<jats:italic>P. aeruginosa<\/jats:italic>mutants defective in virulence factor production implicated cyanide as a key component inhibiting the ciliary beat frequency.<\/jats:p><jats:p>If cyanide production similarly impairs mucocilliary clearance<jats:italic>in vivo<\/jats:italic>, it could explain the link with increased disease severity observed in cystic fibrosis patients with detectable cyanide in their airway.<\/jats:p>","DOI":"10.1183\/09031936.00097014","type":"journal-article","created":{"date-parts":[[2014,9,4]],"date-time":"2014-09-04T13:33:01Z","timestamp":1409837581000},"page":"1253-1261","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":41,"title":["Cyanide levels found in infected cystic fibrosis sputum inhibit airway ciliary 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