ABCMdb

PMID: 23164641

Kelly C, Williams MT, Elborn JS, Ennis M, Schock BC
Expression of the inflammatory regulator A20 correlates with lung function in patients with cystic fibrosis.
J Cyst Fibros. 2013 Jul;12(4):411-5. doi: 10.1016/j.jcf.2012.10.009. Epub 2012 Nov 17., [PubMed]

Sentences

No. Mutations Sentence Comment

4 ABCC7 p.Arg117His 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. Login to comment 7 ABCC7 p.Arg117His 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. Login to comment 27 ABCC7 p.Arg117His Here we further investigate the relationship between A20, TAX1BP1 and CFTR as well as any association with lung function (FEV1) in patients homozygous for F508del and heterozygous for R117H/F508del. Login to comment 29 ABCC7 p.Arg117His Materials and methods Primary nasal epithelial cells (NEC) were obtained from CF patients (F508del/F508del {n=7}, R117H/F508del {n=6}) and age matched controls {n=8} as previously described [11] and were fully differentiated at air-liquid interface (ALI). Login to comment 42 ABCC7 p.Arg117His The effect of different CF genotypes (R117H/F508del and F508del/F508del) on basal A20 (C) and TAX1BP1 (D) mRNA expression was determined by qPCR. Login to comment 58 ABCC7 p.Arg117His F508del homozygotes showed a more pronounced reduction in A20 expression than R117H/F508del heterozygotes (pb0.05, Fig. 1E). Login to comment 60 ABCC7 p.Arg117His To provide a basic indication of how reduced A20 expression may relate to NF-ba;B and the inflammatory response we assessed the expression of p65 (NF-ba;B subunit) by qPCR Following LPS stimulation, p65 expression was higher in F508del homozygotes than R117H/F508del heterozygotes (pb0.05, Fig. 2A). Login to comment 65 ABCC7 p.Arg117His The median FEV1 for the R117H/F508del Fig. 2. Login to comment 67 ABCC7 p.Arg117His The effect of different CF genotypes (R117H/F508del and F508del/ F508del) on LPS-induced p65 (A) expression was determined by qPCR. Login to comment 82 ABCC7 p.Arg117His Basally and following LPS stimulation, A20 expression was reduced in CF cells in a manner that largely reflected genotype; the F508del/F508del group had a lower expression than the R117H/F508del group. Login to comment