Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation

Autophagy

Volumn 10, Issue 11, 2014, Pages 2053-2074

  De Stefano, Daniela ^a   Villella, Valeria R ^a   Esposito, Speranza ^a   Tosco, Antonella ^b   Sepe, Angela ^b   De Gregorio, Fabiola ^b   Salvadori, Laura ^b   Grassia, Rosa ^c   Leone, Carlo A ^c   De Rosa, Giuseppe ^b   Maiuri, Maria C ^b,d   Pettoello Mantovani, Massimo ^e   Guido, Stefano ^b   Bossi, Anna ^f   Zolin, Anna ^f   Venerando, Andrea ^g   Pinna, Lorenzo A ^g   Mehta, Anil ^h   Bona, Gianni ⁱ   Kroemer, Guido ^d,j,k,l   Maiuri, Luigi ^a,i   Raia, Valeria ^b   more..

^a SAN RAFFAELE HOSPITAL   (Italy)

^b UNIVERSITY OF NAPLES FEDERICO II   (Italy)

^c MONALDI HOSPITAL   (Italy)

^d CENTRE DE RECHERCHE DES CORDELIERS   (France)

^e UNIVERSITY OF FOGGIA   (Italy)

^f UNIVERSITY OF MILAN   (Italy)

^g UNIVERSITY OF PADOVA   (Italy)

^h UNIVERSITY OF DUNDEE   (United Kingdom)

ⁱ UNIVERSITY OF EASTERN PIEDMONT   (Italy)

^j UNIVERSITÉ PARIS DESCARTES   (France)

^k INSTITUT GUSTAVE ROUSSY   (France)

^l HÔPITAL EUROPÉEN GEORGES POMPIDOU   (France)

more..

Author keywords

Autophagy; CFTR; Cysteamine; Cystic fibrosis; Epigallocatechin gallate; Sweat chloride

Indexed keywords

BECLIN 1; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EPIGALLOCATECHIN GALLATE; INTERLEUKIN 8; MERCAPTAMINE; PROTEIN P62; TUMOR NECROSIS FACTOR; APOPTOSIS REGULATORY PROTEIN; BECN1 PROTEIN, HUMAN; BECN1 PROTEIN, MOUSE; CATECHIN; CFTR PROTEIN, HUMAN; CHLORIDE; CYSTAMINE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR DELTA F508; MEMBRANE PROTEIN; SIGNAL TRANSDUCING ADAPTOR PROTEIN; SQSTM1 PROTEIN, HUMAN; TUMOR NECROSIS FACTOR ALPHA;

ARTICLE; CELL MEMBRANE; CFTR GENE; CXCL8 GENE; CYSTIC FIBROSIS; GENE EXPRESSION; GENE MUTATION; HOMOZYGOSITY; HUMAN; IN VITRO STUDY; IN VIVO STUDY; MORTALITY; MUTATOR GENE; NONHUMAN; NOSE MUCOSA; PHASE 2 CLINICAL TRIAL (TOPIC); PHENOTYPE; TNF ALPHA GENE; ADOLESCENT; ADULT; ANALOGS AND DERIVATIVES; ANIMAL; CHEMISTRY; CHILD; CLINICAL TRIAL; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR MOUSE; FEMALE; GENETICS; HOMOZYGOTE; MALE; METABOLISM; MOUSE; MUTATION; ORAL DRUG ADMINISTRATION; PHASE 2 CLINICAL TRIAL; PILOT STUDY; TRANSGENIC MOUSE; YOUNG ADULT;

MUS;

ADAPTOR PROTEINS, SIGNAL TRANSDUCING; ADMINISTRATION, ORAL; ADOLESCENT; ADULT; ANIMALS; APOPTOSIS REGULATORY PROTEINS; CATECHIN; CELL MEMBRANE; CHILD; CHLORIDES; CYSTAMINE; CYSTEAMINE; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; FEMALE; HOMOZYGOTE; HUMANS; INTERLEUKIN-8; MALE; MEMBRANE PROTEINS; MICE; MICE, INBRED CFTR; MICE, TRANSGENIC; MUTATION; PHENOTYPE; PILOT PROJECTS; TUMOR NECROSIS FACTOR-ALPHA; YOUNG ADULT;

EID: 84919764939     PISSN: 15548627     EISSN: 15548635     Source Type: Journal    
DOI: 10.4161/15548627.2014.973737     Document Type: Article

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