Abstract
Background: A20 and TAX1BP1 interact to negatively regulate NF-¿B-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 FEV1 in all CF patients (r= 0.968, pb0.001). 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.
Original language | English |
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Pages (from-to) | 411-415 |
Number of pages | 5 |
Journal | Journal of Cystic Fibrosis |
Volume | 12 |
Issue number | 4 |
DOIs | |
Publication status | Published - 31 Jul 2013 |
Keywords
- cystic fibrosis
- A20 protein
- NF kappa B
- airway epithelial cells
- chronic inflamation