Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition

Nature Cell Biology

Volumn 12, Issue 9, 2010, Pages 863-875

  Luciani, Alessandro ^a,b   Villella, Valeria Rachela ^b   Esposito, Speranza ^b,c   Brunetti Pierri, Nicola ^b,d   Medina, Diego ^d   Settembre, Carmine ^d   Gavina, Manuela ^a   Pulze, Laura ^a   Giardino, Ida ^c   Pettoello Mantovani, Massimo ^c   D'Apolito, Maria ^c   Guido, Stefano ^b   Masliah, Eliezer ^e   Spencer, Brian ^e   Quaratino, Sonia ^f   Raia, Valeria ^b   Ballabio, Andrea ^b,d   Maiuri, Luigi ^a,c  

^a SAN RAFFAELE HOSPITAL   (Italy)

^b UNIVERSITY OF NAPLES FEDERICO II   (Italy)

^c UNIVERSITY OF FOGGIA   (Italy)

^d TELETHON INSTITUTE OF GENETICS AND MEDICINE TIGEM   (Italy)

^e UNIVERSITY OF CALIFORNIA   (United States)

^f UNIVERSITY OF SOUTHAMPTON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ANTIOXIDANT; BECLIN 1; CYSTAMINE; GREEN FLUORESCENT PROTEIN; PHOSPHATIDYLINOSITOL 3 KINASE; PROTEIN GLUTAMINE GAMMA GLUTAMYLTRANSFERASE; PROTEIN P62; REACTIVE OXYGEN METABOLITE; TRANSMEMBRANE CONDUCTANCE REGULATOR; UBIQUINOL CYTOCHROME C REDUCTASE; ACETYLCYSTEINE; APOPTOSIS REGULATORY PROTEIN; BECN1 PROTEIN, HUMAN; BECN1 PROTEIN, MOUSE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EPITHELIAL SODIUM CHANNEL; EUK-134; HEAT SHOCK PROTEIN; LIGHT CHAIN 3, HUMAN; MAP1LC3 PROTEIN, MOUSE; MEMBRANE PROTEIN; MICROTUBULE ASSOCIATED PROTEIN; ORGANOMETALLIC COMPOUND; PROTEIN BINDING; SALICYLIC ACID DERIVATIVE; SCNN1B PROTEIN, MOUSE; SIGNAL TRANSDUCING ADAPTOR PROTEIN; SQSTM1 PROTEIN, HUMAN; SQSTM1 PROTEIN, MOUSE; SUMO PROTEIN; TISSUE TRANSGLUTAMINASE 2, HUMAN;

ADOLESCENT; ADULT; AGGRESOME; ANIMAL CELL; ANIMAL TISSUE; ARTICLE; AUTOPHAGY; BRONCHUS MUCOSA; CANCER CELL CULTURE; CELL SURFACE; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; CYSTIC FIBROSIS; ENDOPLASMIC RETICULUM; EPITHELIUM CELL; FEMALE; GENE OVEREXPRESSION; GENE SILENCING; HUMAN; HUMAN CELL; HUMAN TISSUE; IN VITRO STUDY; IN VIVO STUDY; LUNG PARENCHYMA; MALE; MOUSE; NASAL BIOPSY; NONHUMAN; NOSE MUCOSA; OXIDATIVE STRESS; PATHOGENESIS; PHENOTYPE; PNEUMONIA; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN CROSS LINKING; PROTEIN LOCALIZATION; PROTEIN TRANSPORT; SCHOOL CHILD; SIGNAL TRANSDUCTION; TRANSGENIC MOUSE; ANIMAL; ANTAGONISTS AND INHIBITORS; BIOLOGICAL MODEL; CELL LINE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR MOUSE; CYTOLOGY; DRUG EFFECTS; GENETICS; INBRED MOUSE STRAIN; INFLAMMATION; METABOLISM; NASAL POLYPS; PHYSIOLOGY; RESPIRATORY TRACT MUCOSA; YOUNG ADULT;

1-PHOSPHATIDYLINOSITOL 3-KINASE; ACETYLCYSTEINE; ADAPTOR PROTEINS, SIGNAL TRANSDUCING; ADOLESCENT; ADULT; ANIMALS; ANTIOXIDANTS; APOPTOSIS REGULATORY PROTEINS; AUTOPHAGY; CELL LINE; CYSTAMINE; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EPITHELIAL SODIUM CHANNEL; HEAT-SHOCK PROTEINS; HUMANS; INFLAMMATION; MEMBRANE PROTEINS; MICE; MICE, INBRED CFTR; MICE, INBRED STRAINS; MICE, TRANSGENIC; MICROTUBULE-ASSOCIATED PROTEINS; MODELS, BIOLOGICAL; NASAL POLYPS; ORGANOMETALLIC COMPOUNDS; PROTEIN BINDING; PROTEIN TRANSPORT; REACTIVE OXYGEN SPECIES; RESPIRATORY MUCOSA; SALICYLATES; SMALL UBIQUITIN-RELATED MODIFIER PROTEINS; TRANSGLUTAMINASES; YOUNG ADULT; EPITHELIAL SODIUM CHANNELS; PHOSPHATIDYLINOSITOL 3-KINASES;

MUS;

EID: 77956396747     PISSN: 14657392     EISSN: None     Source Type: Journal    
DOI: 10.1038/ncb2090     Document Type: Article

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