Functional features of KIR2DS4 expressed in NK and T cells: Role in pediatric autoimmune hepatitis

A. PODHORZER; G. THEILER; N. PALADINO; M. CUARTEROLO; S.I. LÓPEZ; M. CAPUCCHIO; P. BAZ; L. UDOVIN; A. MACHICOTE; L. FAINBOIM

Congreso; LIX Reunión Científica Anual de la Sociedad Argentina de Investigación Clínica y de la LXII Reunión Científica Anual de la Sociedad Argentina de Inmunología; 2014

Background: Activation of natural killer (NK) cells involves integration of signals from activating and inhibitory receptors. The oldest, most prevalent KIR2DS is KIR2DS4, which is represented by a variable balance between ?full-length? (FL) and ?deleted? forms (KIR1D). In a previous work we have found that KIR2DS4-FL gene frequency was increased showing a very strong synergistic effect with HLA-DRB1*1301 in the susceptibility to develop type I pediatric autoimmune hepatitis (PAH)Aims: Despite its increased frequency, little is known about the functional activity mediated by KIR2DS4.Thus, we explore: 1-The presence of putative KIR2DS4 ligands.2-The presence of inhibitory KIR-HLA-C ligands.3-Evaluate the expression KIR genes between PAH and controls.4-The pattern of cytokine secretion mediated by anti-KIR2DS4 as its ligandResults: Using PCR-SSOP method we observed that HLA-Cw2, one of proposed KIR2DS4 ligands, is increased in PAH (p