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---> Echelle macroscopique
encombrement des voies respiratoires qui diminue les capacités respiratoires des malades et par des infections respiratoires fréquentes = toux
---> Echelle cellulaire
Certaines cellules de l’épithélium pulmonaire, regroupées en glandes, sécrètent un mucus ( une sécrétion
visqueuse) qui tapisse sa surface. obstruction des bronches et des bronchioles par un mucus anormalement épais
Ce mucus retient les agents pathogènes et favorise des infections et des inflammations chroniques
---> Echelle moléculaire
mutation du gène CFTR (elle fabrique la protéine CFTR: canal ionique qui permet le passage d'ions chlorure à travers la membrane des cellules) sur le chromosome 7
élimination de l'acide aminé phénylalanine
les protéines « anormales » synthétisés par les cellules de l’épithélium sont à l’origine d’un mucus visqueux (non fluide)
Ce dysfonctionnement altère la production des sécrétions de différentes cellules de l'organisme, entraînant des atteintes respiratoires (infections) et des troubles digestifs (par atteinte du pancréas).
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