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Mammals also have motile cilia or secondary cilia that are usually present on a cell's surface in large numbers (multiciliate), and beat in coordinated metachronal waves. [40] Multiciliated cells are found lining the respiratory tract where they function in mucociliary clearance sweeping mucus containing debris away from the lungs . [ 13 ]
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]
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]
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 are hair-like, microtubular-based structures on the luminal surface of the epithelium. On each epithelial cell there are around 200 cilia that beat constantly at a rate of between 10 and 20 times per second. The cilia are surrounded by a periciliary liquid layer (PCL), a sol layer that is overlain with the gel layer of mucus. [9]
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Lung diseases due to external agents (1 C, 20 P) P. Pneumonia (2 C, 50 P) R. Respiratory diseases principally affecting the interstitium (8 P) S. Syndromes affecting ...