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10.1038/ncomms10791

http://scihub22266oqcxt.onion/10.1038/ncomms10791
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suck abstract from ncbi


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pmid26972847
      Nat+Commun 2016 ; 7 (ä): 10791
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  • IL-1 receptor antagonist ameliorates inflammasome-dependent inflammation in murine and human cystic fibrosis #MMPMID26972847
  • Iannitti RG ; Napolioni V ; Oikonomou V ; De Luca A ; Galosi C ; Pariano M ; Massi-Benedetti C ; Borghi M ; Puccetti M ; Lucidi V ; Colombo C ; Fiscarelli E ; Lass-Flörl C ; Majo F ; Cariani L ; Russo M ; Porcaro L ; Ricciotti G ; Ellemunter H ; Ratclif L ; De Benedictis FM ; Talesa VN ; Dinarello CA ; van de Veerdonk FL ; Romani L
  • Nat Commun 2016[Mar]; 7 (ä): 10791 PMID26972847 show ga
  • Dysregulated inflammasome activation contributes to respiratory infections and pathologic airway inflammation. Through basic and translational approaches involving murine models and human genetic epidemiology, we show here the importance of the different inflammasomes in regulating inflammatory responses in mice and humans with cystic fibrosis (CF), a life-threatening disorder of the lungs and digestive system. While both contributing to pathogen clearance, NLRP3 more than NLRC4 contributes to deleterious inflammatory responses in CF and correlates with defective NLRC4-dependent IL-1Ra production. Disease susceptibility in mice and microbial colonization in humans occurs in conditions of genetic deficiency of NLRC4 or IL-1Ra and can be rescued by administration of the recombinant IL-1Ra, anakinra. These results indicate that pathogenic NLRP3 activity in CF could be negatively regulated by IL-1Ra and provide a proof-of-concept evidence that inflammasomes are potential targets to limit the pathological consequences of microbial colonization in CF.
  • |Adolescent [MESH]
  • |Adult [MESH]
  • |Animals [MESH]
  • |Apoptosis Regulatory Proteins/genetics/immunology [MESH]
  • |Aspergillosis/*immunology [MESH]
  • |Aspergillus fumigatus [MESH]
  • |Autophagy/genetics/immunology [MESH]
  • |Blotting, Western [MESH]
  • |CARD Signaling Adaptor Proteins/genetics/immunology [MESH]
  • |Calcium-Binding Proteins/genetics/immunology [MESH]
  • |Carrier Proteins/genetics/immunology [MESH]
  • |Cell Line [MESH]
  • |Child [MESH]
  • |Child, Preschool [MESH]
  • |Cystic Fibrosis Transmembrane Conductance Regulator/genetics [MESH]
  • |Cystic Fibrosis/genetics/*immunology/microbiology [MESH]
  • |Cytokines/*genetics/immunology [MESH]
  • |Disease Models, Animal [MESH]
  • |Enzyme-Linked Immunosorbent Assay [MESH]
  • |Epithelial Cells/*immunology [MESH]
  • |Female [MESH]
  • |Fluorescent Antibody Technique [MESH]
  • |Humans [MESH]
  • |Immunohistochemistry [MESH]
  • |In Situ Nick-End Labeling [MESH]
  • |Infant [MESH]
  • |Inflammasomes/*genetics/immunology [MESH]
  • |Inflammation [MESH]
  • |Interleukin 1 Receptor Antagonist Protein/*genetics/immunology/pharmacology [MESH]
  • |Lung/*metabolism [MESH]
  • |Macrophages/immunology [MESH]
  • |Male [MESH]
  • |Mice [MESH]
  • |Mice, Inbred C57BL [MESH]
  • |Mice, Knockout [MESH]
  • |Middle Aged [MESH]
  • |NLR Family, Pyrin Domain-Containing 3 Protein [MESH]
  • |Polymorphism, Single Nucleotide [MESH]
  • |Pseudomonas Infections/*immunology [MESH]
  • |Pseudomonas aeruginosa [MESH]
  • |Respiratory Mucosa/cytology [MESH]
  • |Reverse Transcriptase Polymerase Chain Reaction [MESH]


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