Cilia from a cystic fibrosis patient react to the ciliotoxicPseudomonas aeruginosaII lectin in a similar manner to normal control cilia – a case report

1997 ◽  
Vol 111 (8) ◽  
pp. 760-762 ◽  
Author(s):  
Elizabeth C. Adam ◽  
Dietlind U. Schumacher ◽  
Udo Schumacher

AbstractThe ciliary beat frequency measurements taken from a nasal polyp from a cystic fibrosis patient were similar to that of the control nasal polyps. The addition of a ciliotoxic lectin produced byPseudomonas aeruginosastopped the beating of the cilia as in the controls. This reaction could be blocked by the pre-incubation of the lectin with its inhibitor fucose. As in the control, the addition of fucose after the cilia had slowed resulted in a return to normal ciliary beating within 24 hours. This shows that the delta F508 CF mutation observed in this patient does not affect ciliary beating and suggests that treatment with fucose in the early stages of aPseudomonas aeruginosainfection could be advantageous for cystic fibrosis patients.

2017 ◽  
Author(s):  
M. Chioccioli ◽  
L. Feriani ◽  
J. Kotar ◽  
P. E. Bratcher ◽  
P. Cicuta

AbstractThe diagnosis and treatment of respiratory disorders are challenging and would benefit from new approaches to systematically assess ciliary beating dynamics and to test new drugs. A novel approach based on multiscale differential dynamic microscopy (multi-DDM) is shown to quantitatively assess collective beating of cilia in a non-biased automated manner, in human airway epithelial cells (HAECs) derived from subjects with cystic fibrosis (CF) and grown in 2D air-liquid interface culture. Multi-DDM can readily detect changes in both ciliary beat frequency (CBF) and cilia coordination that result from perturbations to the mucosal layer. The efficacy of three CFTR-modulating treatments is investigated: ivacaftor (VX-770) with lumacaftor (VX-809), VX-809 alone and Thymosin alpha 1 (Tα1) alone. All three treatments restore coordination of cilia beating in the CF cells, albeit to varying degrees. We argue cilia are affected by these treatments through the physical properties of the mucus. Phenotyping cilia dynamics through multi-DDM provides novel insight into the response of ciliary beating following treatment with drugs, and has application in the broader context of respiratory disease and for drug screening.One sentence summaryA semi-automated and unbiased assay based on multiscale differential dynamic microscopy (multi-DDM) detects changes in the coordination and frequency of ciliary beating in F508del/F508del primary human airway cells under different conditions and in response to CFTR-modulating compounds.


2001 ◽  
Vol 280 (6) ◽  
pp. C1485-C1497 ◽  
Author(s):  
Diane M. Morse ◽  
Jennifer L. Smullen ◽  
C. William Davis

The purinergic regulation of ciliary activity was studied using small, continuously superfused explants of human nasal epithelium. The P2Y2 purinoceptor (P2Y2-R) was identified as the major purinoceptor regulating ciliary beat frequency (CBF); UTP (EC50 = 4.7 μM), ATP, and adenosine-5′- O-(3-thiotriphosphate) elicited similar maximal responses, approximately twofold over baseline. ATP, however, elicited a post-peak sustained plateau in CBF (1.83 ± 0.1-fold), whereas the post-peak CBF response to UTP declined over 15 min to a low-level plateau (1.36 ± 0.16-fold). UDP also stimulated ciliary beating, probably via P2Y6-R, with a maximal effect approximately one-half that elicited by P2Y2-R stimulation. Not indicated were P2Y1-R-, P2Y4-R-, or P2Y11-R-mediated effects. A2B-receptor agonists elicited sustained responses in CBF approximately equal to those from UTP/ATP [5′-( N-ethylcarboxamido)adenosine, EC50 = 0.09 μM; adenosine, EC50 = 0.7 μM]. Surprisingly, ADP elicited a sustained stimulation in CBF. The ADP effect and the post-peak sustained portion of the ATP response in CBF were inhibited by the A2-R antagonist 8-( p-sulfophenyl)theophylline. Hence, ATP affects ciliary activity through P2Y2-R and, after an apparent ectohydrolysis to adenosine, through A2BAR.


2016 ◽  
Vol 16 (1) ◽  
Author(s):  
Daniel Goldenberger ◽  
Vladimira Hinić ◽  
Spasenija Savic Prince ◽  
Michael Tamm ◽  
Anna-Maria Balestra ◽  
...  

1988 ◽  
Vol 56 (3) ◽  
pp. 665-672 ◽  
Author(s):  
B L Pasloske ◽  
A M Joffe ◽  
Q Sun ◽  
K Volpel ◽  
W Paranchych ◽  
...  

2017 ◽  
Author(s):  
Csaba Verasztó ◽  
Nobuo Ueda ◽  
Luis A. Bezares-Calderón ◽  
Aurora Panzera ◽  
Elizabeth A. Williams ◽  
...  

AbstractCiliated surfaces harbouring synchronously beating cilia can generate fluid flow or drive locomotion. In ciliary swimmers, ciliary beating, arrests, and changes in beat frequency are often coordinated across extended or discontinuous surfaces. To understand how such coordination is achieved, we studied the ciliated larvae of Platynereis dumerilii, a marine annelid. Platynereis larvae have segmental multiciliated cells that regularly display spontaneous coordinated ciliary arrests. We used whole-body connectomics, activity imaging, transgenesis, and neuron ablation to characterize the ciliomotor circuitry. We identified cholinergic, serotonergic, and catecholaminergic ciliomotor neurons. The synchronous rhythmic activation of cholinergic cells drives the coordinated arrests of all cilia. The serotonergic cells are active when cilia are beating. Serotonin inhibits the cholinergic rhythm, and increases ciliary beat frequency. Based on their connectivity and alternating activity, the catecholaminergic cells may generate the rhythm. The ciliomotor circuitry thus constitutes a stop-and-go pacemaker system for the whole-body coordination of ciliary locomotion.


2020 ◽  
Vol 9 (26) ◽  
Author(s):  
Andrea Sass ◽  
Tom Coenye

ABSTRACT Pseudomonas aeruginosa is an opportunistic pathogen that is able to cause various infections, including airway infections in cystic fibrosis patients. Here, we present the complete closed and annotated genome sequence of P. aeruginosa AA2, an isolate obtained early during infection of the respiratory tract of a German cystic fibrosis patient.


1988 ◽  
Vol 65 (4) ◽  
pp. 1617-1620 ◽  
Author(s):  
D. R. Maurer ◽  
J. Liebman

Consumption of ethanol can impair lung function and slow total lung clearance. High concentrations of ethanol have been shown to slow or arrest ciliary beating. This study examined the effects of concentrations of alcohol comparable to blood levels achieved from social drinking on ciliary beat frequency. We obtained ciliated cells by brushing the trachea of unanesthetized sheep during fiber-optic bronchoscopy. The cells were suspended in a perfusion chamber and physiological conditions were maintained in vitro. Ciliary beat frequency and synchrony were determined by slow-motion analysis of video images obtained by interference contrast microscopy. Metachronal ciliary coordination was observed in all preparations. The ciliary beat frequency was stimulated at ethanol concentrations from 0.01 up to but not including 0.1%, unchanged at 0.5 and 1%, and slowed at 2%. While confirming inhibition of ciliary motility at very high ethanol levels, we observed no acute impairment of ciliary function at ethanol concentrations comparable to those achieved from social drinking. Indeed, we found an unexpected stimulation of ciliary beating at low levels of ethanol. How this alteration in ciliary beating would affect pulmonary clearance remains unknown at this time.


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