Immotile cilia syndrome

A defect or absence in any one of the proteins within the axoneme (e.g. dynein, nexin, etc.) results in cilia that are immotile, so called immotile-cilia syndrome. Patients suffering from this disease have chronic pulmonary disorders due to the cilia in the respiratory tract being unable to sweep out bacteria and other foreign particles. In addition, males with this genetic defect are infertile because their sperm are unable to move due to flagella inactivity. 

Some people have defective dynein, which causes an immotile-cilia syndrome. This leads to chronic respiratory disorders and also infertility in males. Explain. 

Afzelius, B.A. (1985). The immotile-cilia syndrome a microtubule-associated defect. CRC Crit. Rev. Biochem. 19, 63-87. 

Contrary to the situation in the usual types of immotile cilia syndrome, the proportion of ciliated... 

Ruusa J, Svartengren M, Philipson K, Camner P. Traeheobronehial particle deposition and elearanee in immotile cilia syndrome patients. J Aerosol Med 1993 6 89 98. 

Mossberg B, Afzelius BA, Eliasson R, Camner P. On the pathogenesis of obstructive lung disease. A study on the immotile-cilia syndrome. Seand J Respir Dis 1978 59 55-65. 

Svartengren K, Philipson K, Svartengren M, Nerbrink O, Camner P. Clearanee in smaller airways of inhaled 6- 0.m particles in subjects with immotile-cilia syndrome. Exp Lung Res 1995 21 667-682. 

Particle clearance by cough supports mucociliary clearance, especially under pathophysiological conditions of diseased lungs (60), such as chronic obstructive pulmonary disease (COPD), immotile cilia syndrome (ICS), and cystic fibrosis (CF). An increased secretion in the airways seems to be necessary. During cough, mucus is propelled toward the larynx by the very fast exhalation, with linear air... 

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