Lack of GAS2L2 Causes PCD by Impairing Cilia Orientation and Mucociliary Clearance.

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第一作者： Ximena M,Bustamante-Marin

第一单位： Cystic Fibrosis/Pulmonary Research and Treatment Center, University of North Carolina, Chapel Hill, NC 27599, USA.

作者： Ximena M,Bustamante-Marin ^[1] ; Wei-Ning,Yin ^[1] ; Patrick R,Sears ^[1] ; Michael E,Werner ^[2] ; Eva J,Brotslaw ^[3] ; Brian J,Mitchell ^[3] ; Corey M,Jania ^[4] ; Kirby L,Zeman ^[5] ; Troy D,Rogers ^[1] ; Laura E,Herring ^[6] ; Luc,Refabért ^[7] ; Lucie,Thomas ^[8] ; Serge,Amselem ^[8] ; Estelle,Escudier ^[8] ; Marie,Legendre ^[8] ; Barbara R,Grubb ^[1] ; Michael R,Knowles ^[1] ; Maimoona A,Zariwala ^[9] ; Lawrence E,Ostrowski ^[10]

作者单位： Cystic Fibrosis/Pulmonary Research and Treatment Center, University of North Carolina, Chapel Hill, NC 27599, USA. ^[1] Biology Department, University of North Carolina, Chapel Hill, NC 27599, USA. ^[2] Department of Cell and Molecular Biology, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA. ^[3] Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of North Carolina, Chapel Hill, NC 27599, USA. ^[4] Center for Environmental Medicine, Asthma, and Lung Biology, University of North Carolina, Chapel Hill, NC 27599, USA. ^[5] Department of Pharmacology, University of North Carolina, Chapel Hill, NC 27599, USA. ^[6] Assistance Publique-Hôpitaux de Paris, Service de Pneumologie et Allergologie Pédiatriques, Hôpital Necker-Enfants Malades, Université Paris Descartes, Paris 75015, France. ^[7] Sorbonne Université, Institut National de la Santé et de la Recherche Médicale, Childhood Genetic Disorders, Département de Génétique Médicale, Assistance Publique-Hôpitaux de Paris, Hôpital Trousseau, Paris 75012, France. ^[8] Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, NC 27599, USA. Electronic address: maimoona_zariwala@med.unc.edu. ^[9] Cystic Fibrosis/Pulmonary Research and Treatment Center, University of North Carolina, Chapel Hill, NC 27599, USA. Electronic address: ostro@med.unc.edu. ^[10]

关键词 GAS2L2 MCC PCD ciliary orientation mucociliary clearance primary ciliary dyskinesia