Decreased expression of HLA-DQ and HLA-DR on cells of the monocytic lineage in cystic fibrosis

摘要: We studied HLA class II molecules on blood monocyte subsets, dendritic cells, sputum macrophages, and monocyte-derived macrophages at the protein (flow cytometry) mRNA level (RT-PCR) in adult patients with cystic fibrosis (CF) healthy control subjects as putative contributors to CF phenotype. In donors, we found a high average HLA-DQ expression of 4.35 mean specific fluorescence intensity units (ΔMnI) classical monocytes. F508del homozygous patients, ΔMnI was low (1.80). Patients were divided into two groups, which 14 these had above 2 (average 3.25 ΔMnI, CF-DQgroup1) 36 below 1.24 CF-DQgroup2). Also, CD16-positive subset cells showed much lower levels for CF-DQgroup2 compared controls. from derived monocytes, vitro dramatically decreased background CF-DQgroup2. MHC transcripts reduced sevenfold decrease HLA-DQβ1 patients. Higher inflammation marker CRP associated expression, treatment inflammatory molecule lipopolysaccharide expression. Interferon γ (IFNγ) could overcome this effect donor while, CF, IFNγ-induced activation impaired. Our data demonstrate pronounced reduction is response IFNγ. • show MHCII monocytes macrophages. HLA-DR transcript are also patient C-reactive correlate Reduced appears be linked inflammation. exhibit an impaired IFNgamma.