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Archives de pédiatrie
Volume 23, n° 12S
pages 1247-1253 (décembre 2016)
Doi : 10.1016/S0929-693X(17)30062-3
Nouvelles thérapeutiques ciblant le canal chlorure dans la mucoviscidose
New therapeutic developments in cystic fibrosis
 

Figure 1




Figure 1 : 

Mécanismes physiologiques: synthèse, maturation/migration intracellulaire et fonction de la protéine CFTR.


Figure 2




Figure 2 : 

Symptômes en fonction de la proportion de protéine CFTR active. Combien faut-il de CFTR?

3o% d’activité CFTR est associée à une réduction des symptômes.


Figure 3




Figure 3 : 

Classes de mutations du gène CFTR , conséquences physiopathologiques et cibles thérapeutiques.

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