ciliary beating recovery in deficient human airway epithelial cells after lentivirus ex vivo gene therapy缺乏人工气道上皮细胞纤毛击败复苏后慢病毒体外基因疗法.pdfVIP

Ciliary Beating Recovery in Deficient Human Airway Epithelial Cells after Lentivirus Ex Vivo Gene Therapy 1 2,3,4 5 1 6,7 Brigitte Chhin , Didier Negre , Olivier Merrot , Jacqueline Pham , Yves Tourneur , Denis 7 8 8 ¨ 2,3,4 1,9,10 Ressnikoff , Martine Jaspers , Mark Jorissen , Franc¸ois-Loıc Cosset , Patrice Bouvagnet * ´ ´ ´ 1 Universite de Lyon, Lyon, France, 2 Universite de Lyon (UCB-Lyon1), IFR128, Lyon, France, 3 INSERM, U758, Lyon, France, 4 Ecole Normale Superieure de Lyon, Lyon, ˆ France, 5 Hospices Civils de Lyon, Hopital de la Croix-Rousse, Service ORL, Lyon, France, 6 INSERM, UMR886, Cardioprotection, Lyon, France, 7 Centre Commun de ´ ´ Quantimetrie, Universite de Lyon, Lyon, France, 8 Ear, Nose, and Throat Department, Head and Neck Surgery, Leuven, Belgium, 9 Hospices Civils de Lyon, Groupe ´ ´ ˆ Hospitalier Est, Laboratoire Cardiogenetique, Bron, France, 10 INSERM, CIC 201, Hopital Louis Pradel, Bron, France Abstract Primary Ciliary Dyskinesia is a heterogeneous genetic disease that is characterized by cilia dysfunction of the epithelial cells lining the respiratory tracts, resulting in recurrent respiratory tract infections. Despite lifelong physiological therapy and