%0 Journal Article
%A Castaño-Nuñez, Angel
%A Montes-Cano, Marco-Antonio
%A Garcia-Lozano, Jose-Raul
%A Ortego-Centeno, Norberto
%A Garcia-Hernandez, Francisco-Jose
%A Espinosa, Gerard
%A Graña-Gil, Genaro
%A Sanchez-Burson, Juan
%A Julia, Maria-Rosa
%A Solans, Roser
%A Blanco, Ricardo
%A Barnosi-Marin, Ana-Celia
%A Gomez-de-la-Torre, Ricardo
%A Fanlo, Patricia
%A Rodriguez-Carballeira, Monica
%A Rodriguez-Rodriguez, Luis
%A Camps, Teresa
%A Castañeda, Santos
%A Alegre-Sancho, Juan-Jose
%A Martin, Javier
%A Gonzalez-Escribano, Maria-Francisca
%T Association of Functional Polymorphisms of KIR3DL1/DS1 With Behçet's Disease.
%D 2019
%U http://hdl.handle.net/10668/14846
%X Behçet's disease (BD) is an immune-mediated vasculitis related to imbalances between the innate and adaptive immune response. Infectious agents or environmental factors may trigger the disease in genetically predisposed individuals. HLA-B51 is the genetic factor stronger associated with the disease, although the bases of this association remain elusive. NK cells have also been implicated in the etiopathogenesis of BD. A family of NK receptors, Killer-cell Immunoglobulin-like Receptor (KIR), with a very complex organization, is very important in the education and control of the NK cells by the union to their ligands, most of them, HLA class I molecules. This study aimed to investigate the contribution of certain KIR functional polymorphisms to the susceptibility to BD. A total of 466 BD patients and 444 healthy individuals were genotyped in HLA class I (A, B, and C). The set of KIR genes and the functional variants of KIR3DL1/DS1 and KIR2DS4 were also determined. Frequency of KIR3DL1*004 was lower in patients than in controls (0.15 vs. 0.20, P = 0.005, Pc = 0.015; OR = 0.70; 95% CI 0.54-0.90) in both B51 positive and negative individuals. KIR3DL1*004, which encodes a misfolded protein, is included in a common telomeric haplotype with only one functional KIR gene, KIR3DL2. Both, KIR3DL1 and KIR3DL2 sense pathogen-associated molecular patterns but they have different capacities to eliminate them. The education of the NK cells depending on the HLA, the balance of KIR3DL1/KIR3DL2 licensed NK cells and the different capacities of these receptors to eliminate pathogens could be involved in the etiopathogenesis of BD.
%K Behçet's disease
%K HLA
%K KIR
%K NK cells
%K Functional polymorphisms
%K Área de Gestión Sanitaria Sur de Sevilla
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