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The genetics of fluttering cilia
3/17/11
The movement of the vibrant cilia which line our respiratory airways enables inhaled impurities to be eliminated. In people suffering from primary ciliary dyskinesia (PCD), the structures of these cilia have anomalies which bring about chronic respiratory problems. Within the context of a European project, researchers at the University of Liège are studying this disease in bobtails (Old English sheepdogs), a breed of dog subject to PCD, in order to bring to light the genes which are responsible.
Often termed ‘man’s best friend’ because of its loyalty, the services it can provide humanity or quite simply its companionship, the dog has for a long time occupied an important place at the side of human beings. In effect, besides the qualities we had already recognised in it, the dog is proving to be a particularly interesting model for research into diseases which occur in both it and humanity. That is the case for primary ciliary dyskinesia (PCD). ‘This hereditary disease, which manifests itself through chronic respiratory problems is observed in both humans and various animals, notably dogs, and more specifically in certain specific breeds,’ adds Professor Michel Georges, Director of the Unit of Animal Genomics at the ULg’s GIGA-Research Unit.
In Belgium around a hundred cases of primary ciliary dyskinesia are detected each year. Over the course of time, whilst the disease develops, the chronic respiratory problems of the patients become worse and worse. They are due to a poor functioning of the body’s vibrant cilia, and in particular the cilia of the cells which line the respiratory system. The latter are covered with mucus and their movement forms a kind of conveyor belt called the ‘mucocilliary escalator.’ ‘This escalator takes back up the fluids which line our respiratory airways and which keep in check inhaled dirt. When it doesn’t function, this dirt accumulates and that causes secondary infections,’ explain the researchers. At the present time no treatment allows the primary lesions to be cured. The only thing doctors can do for the patient is to alleviate the symptoms, which are very incapacitating on a daily basis.
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