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Primary ciliary dyskinesia (PCD) is a rare, autosomal recessive genetic ciliopathy, that causes defects in the action of cilia lining the upper and lower respiratory tract, sinuses, Eustachian tube, middle ear, fallopian tube, and flagella of sperm cells.
The cells in the respiratory epithelium are of five main types: a) ciliated cells, b) goblet cells, c) brush cells, d) airway basal cells, and e) small granule cells (NDES) [6] Goblet cells become increasingly fewer further down the respiratory tree until they are absent in the terminal bronchioles; club cells take over their role to some extent here. [7]
The cilia need to be able to move freely in the periciliary liquid layer and when this is impaired through damage to the cilia or by imbalances in the moisture or pH of the PCL, the mucus is unable to be cleared properly from the airways. Cystic fibrosis is a consequence of imbalances in the PCL. [9]
The hyperplasia of airway basal cells is the earliest indication of smoking-related abnormality in the lung. [4] [1] This is followed by shortened cilia, loss of ciliated cells, mucous cell hyperplasia, and loss of cell junctions giving a leaky epithelial barrier. With persistent stress from smoking, the basal cells become disarranged and lose ...
Cilia Structure. Primary cilia are found to be formed when a cell exits the cell cycle. [2] Cilia consist of four main compartments: the basal body at the base, the transition zone, the axenome which is an arrangement of nine doublet microtubules and considered to be the core of the cilium, and the ciliary membrane. [2]
The lungs are the largest organs in the lower respiratory tract. The lungs are suspended within the pleural cavity of the thorax. The pleurae are two thin membranes, one cell layer thick, which surround the lungs. The inner (visceral pleura) covers the lungs and the outer (parietal pleura) lines the inner surface of the chest wall. This ...
A ciliopathy is any genetic disorder that affects the cellular cilia or the cilia anchoring structures, the basal bodies, [1] or ciliary function. [2] Primary cilia are important in guiding the process of development, so abnormal ciliary function while an embryo is developing can lead to a set of malformations that can occur regardless of the particular genetic problem. [3]
The development of bronchiectasis requires two factors: an initial injury to the lung (such as from infection, auto-immune destruction of lung tissue, or other destruction of lung tissue (as seen in gastroesophageal reflux disease or aspiration syndromes)) which leads to impaired mucociliary clearance, obstruction, or a defect in host defense.