Pharmacological Activation of CFTR Function improves Airway Epithelial Homeostasis and Immune Defense in Children with Cystic Fibrosis

被引：0
|
作者：
Loske, J. [1 ,2 ]
Voeller, M. [3 ]
Lukassen, S. [4 ]
Stahl, M. [3 ,5 ,6 ]
Thuermann, L. [1 ]
Seegebarth, A. [1 ]
Roehmel, J. [3 ,5 ,6 ]
Wisniewski, S. [3 ]
Messingschlager, M. [1 ,2 ]
Lorenz, S. [7 ]
Klages, S. [7 ]
Eils, R. [4 ,6 ,8 ]
Lehmann, I. [1 ,5 ,6 ]
Mall, M. [3 ,5 ,6 ]
Trump, S. [1 ]
Graeber, S. Y. [3 ,5 ,6 ]
机构：
[1] Berliner Inst Gesundheitsforsch Charite, Forsch Grp Mol Epidemiol, Berlin, Germany
[2] Free Univ Berlin, Inst Biol, Berlin, Germany
[3] Charite, Klin Padiatr Pneumol Immunol & Intensivmed, Berlin, Germany
[4] Berliner Inst Gesundheitsforsch Charite, Ctr Digital Hlth, Berlin, Germany
[5] Berliner Inst Gesundheitsforsch Charite, Berlin, Germany
[6] Deutsch Zentrum Lungenforsch DZL, Assoziertes Zentrum, Berlin, Germany
[7] Max Planck Inst Mol Genet, Berlin, Germany
[8] Univ Klinikum Heidelberg, Hlth Data Sci Unit, Heidelberg, Germany
来源：
KLINISCHE PADIATRIE | 2024年 / 236卷 / 02期
关键词：
D O I：
10.1055/s-0044-1779396
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
0050
引用
收藏
页码：S23 / S23
页数：1
相关论文
共 50 条
  • [41] CYSTIC-FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR (CFTR) - LOCALIZATION IN SURFACE-MEMBRANES OF AIRWAY EPITHELIAL-CELLS
    XU, J
    HARSCH, AD
    GLICK, MC
    ZASLOFF, MA
    BEVINS, CL
    ZEITLIN, PL
    SCANLIN, TF
    [J]. FASEB JOURNAL, 1991, 5 (04): : A463 - A463
  • [42] Impact of CFTR Modulators on Beta-Cell Function in Children and Young Adults with Cystic Fibrosis
    Piona, Claudia
    Mozzillo, Enza
    Tosco, Antonella
    Volpi, Sonia
    Rosanio, Francesco Maria
    Cimbalo, Chiara
    Franzese, Adriana
    Raia, Valeria
    Zusi, Chiara
    Emiliani, Federica
    Boselli, Maria Linda
    Trombetta, Maddalena
    Bonadonna, Riccardo Crocina
    Cipolli, Marco
    Maffeis, Claudio
    [J]. JOURNAL OF CLINICAL MEDICINE, 2022, 11 (14)
  • [43] CORRECTIVE EFFECTS ON AIRWAY EPITHELIAL FUNCTION BY HUMAN AMNIOTIC EPITHELIAL CELLS (HAEC) WHEN TREATING CYSTIC FIBROSIS
    Kicic, A.
    Ling, K.
    Martinovich, K.
    Garratt, L. W.
    Looi, K.
    Kicic-Starcevich, E.
    Iosifidis, T.
    Sutanto, E. N.
    Tarran, R.
    Cmielewski, P.
    Donnelley, M.
    Parsons, D.
    Stick, S. M.
    Moodley, Y.
    [J]. PEDIATRIC PULMONOLOGY, 2015, 50 : 274 - 274
  • [44] Airway microbial diversity is decreased in young children with cystic fibrosis compared to healthy controls but improved with CFTR modulation
    Hahn, Andrea
    Burrell, Aszia
    Ansusinha, Emily
    Peng, Diane
    Chaney, Hollis
    Sami, Iman
    Perez, Geovanny F.
    Koumbourlis, Anastassios C.
    McCarter, Robert
    Freishtat, Robert J.
    Crandall, Keith A.
    Zemanick, Edith T.
    [J]. HELIYON, 2020, 6 (06)
  • [45] EFFICIENCY OF GENE-TRANSFER FOR RESTORATION OF NORMAL AIRWAY EPITHELIAL FUNCTION IN CYSTIC-FIBROSIS
    JOHNSON, LG
    OLSEN, JC
    SARKADI, B
    MOORE, KL
    SWANSTROM, R
    BOUCHER, RC
    [J]. NATURE GENETICS, 1992, 2 (01) : 21 - 25
  • [46] SFPQ rescues F508del-CFTR expression and function in cystic fibrosis bronchial epithelial cells
    Kumar, Parameet
    Soni, Dharmendra Kumar
    Sen, Chaitali
    Larsen, Mads B.
    Mazan-Mamczarz, Krystyna
    Piao, Yulan
    De, Supriyo
    Gorospe, Myriam
    Frizzell, Raymond A.
    Biswas, Roopa
    [J]. SCIENTIFIC REPORTS, 2021, 11 (01)
  • [47] SFPQ rescues F508del-CFTR expression and function in cystic fibrosis bronchial epithelial cells
    Parameet Kumar
    Dharmendra Kumar Soni
    Chaitali Sen
    Mads B. Larsen
    Krystyna Mazan-Mamczarz
    Yulan Piao
    Supriyo De
    Myriam Gorospe
    Raymond A. Frizzell
    Roopa Biswas
    [J]. Scientific Reports, 11
  • [48] CFTR channels and wound healing. Focus on "Cystic fibrosis transmembrane conductance regulator is involved in airway epithelial wound repair"
    Kirk, Kevin L.
    [J]. AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY, 2010, 299 (05): : C888 - C890
  • [49] CFTR channel: A regulator of cytosolic calcium [Ca2+](i) in normal and cystic fibrosis human airway epithelial cells
    Walsh, D
    Urbach, V
    Harvey, B
    [J]. JOURNAL OF PHYSIOLOGY-LONDON, 1997, 499P : P4 - P4
  • [50] CFTR targeting by interference RNA to generate a cystic fibrosis phenotype in well-polarized human airway epithelial cells.
    Bellec, Jessica
    Losa, Davide
    Bacchetta, Marc
    Chanson, Marc
    Tuan Huy Nguyen
    [J]. HUMAN GENE THERAPY, 2012, 23 (10) : A83 - A83
12345