title: Expression of the inflammatory regulator A20 correlates with lung function in patients with cystic fibrosis.
abstract: BACKGROUND
  A20 and TAX1BP1 interact to negatively regulate NF-κB-driven inflammation. A20 expression is altered in F508del/F508del patients. Here we explore the effect of CFTR and CFTR genotype on A20 and TAX1BP1 expression. The relationship with lung function is also assessed.
  
  
  METHODS
  Primary nasal epithelial cells (NECs) from CF patients (F508del/F508del, n=7, R117H/F508del, n=6) and controls (age-matched, n=8), and 16HBE14o- cells were investigated. A20 and TAX1BP1 gene expression was determined by qPCR.
  
  
  RESULTS
  Silencing of CFTR reduced basal A20 expression. Following LPS stimulation A20 and TAX1BP1 expression was induced in control NECs and reduced in CF NECs, broadly reflecting the CF genotype: F508del/F508del had lower expression than R117H/F508del. A20, but not TAX1BP1 expression, was proportional to FEV(1) in all CF patients (r=0.968, p<0.001).
  
  
  CONCLUSIONS
  A20 expression is reduced in CF and is proportional to FEV1. Pending confirmation in a larger study, A20 may prove a novel predictor of CF inflammation/disease severity.
authors.affiliation: Centre for Infection and Immunity, Queen's University of Belfast, Belfast BT97AE, UK.
authors.forename: Catriona
authors.initials: C
authors.lastname: Kelly
authors.forename: Mark T
authors.initials: MT
authors.lastname: Williams
authors.forename: J Stuart
authors.initials: JS
authors.lastname: Elborn
authors.forename: Madeleine
authors.initials: M
authors.lastname: Ennis
authors.forename: Bettina C
authors.initials: BC
authors.lastname: Schock
mesh_terms: D000328:Adult; D002478:Cells, Cultured; D003550:Cystic Fibrosis / Q000150:complications / Q000235:genetics* / Q000378:metabolism / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D004268:DNA-Binding Proteins / Q000096:biosynthesis / Q000235:genetics*; D005260:Female; D005541:Forced Expiratory Volume; D015870:Gene Expression; D006801:Humans; D007249:Inflammation / Q000209:etiology / Q000235:genetics; D047908:Intracellular Signaling Peptides and Proteins / Q000096:biosynthesis / Q000235:genetics*; D008297:Male; D009363:Neoplasm Proteins / Q000235:genetics; D009687:Nuclear Proteins / Q000096:biosynthesis / Q000235:genetics*; D000072598:Tumor Necrosis Factor alpha-Induced Protein 3; D055815:Young Adult
chemical_list: D004268:DNA-Binding Proteins; D047908:Intracellular Signaling Peptides and Proteins; D009363:Neoplasm Proteins; D009687:Nuclear Proteins; C120635:TAX1BP1 protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; C076529:TNFAIP3 protein, human; D000072598:Tumor Necrosis Factor alpha-Induced Protein 3