Natural killer (NK) cells are essential for the elimination of the transformed and cancerous cells. Killer cell immunoglobulin-like receptors (KIRs) which expressed by T and NK cells, are key regulator of NK cell function. The KIR and their ligands, MHC class I (HLA-A, B and C) molecules, are highly polymorphic and their related genes are located on 19 q13.4 and 6 q21.3 chromosomes, respectively. It is clear that particular interaction between the KIRs and their related ligands can influence on the prevalence, progression and outcome of several diseases, like complications of pregnancy, viral infection, autoimmune diseases, and hematological malignancies. The mechanisms of immune signaling in particular NK cells involvement in causing pathological conditions are not completely understood yet. Therefore, better understanding of the molecular mechanism of KIR-MHC class I interaction could facilitate the treatment strategy of diseases. The present review focused on the main characteristics and functional details of various KIR and their combination with related ligands in diseases and also highlights ongoing efforts to manipulate the key checkpoints in NK cell-based immunotherapy.

天然杀伤（NK）细胞对于消除转化的癌细胞是必不可少的。由T细胞和NK细胞表达的杀伤细胞免疫球蛋白样受体（KIR）是NK细胞功能的关键调节因子。KIR及其配体MHC I类（HLA-A，B和C）分子具有高度多态性，其相关基因分别位于19 q13.4和6 q21.3染色体上。显然，KIR及其相关配体之间的特殊相互作用会影响多种疾病的发生，进展和结果，如妊娠并发症，病毒感染，自身免疫性疾病和血液系统恶性肿瘤。尚未完全了解免疫信号传导的机制，特别是NK细胞参与引起病理状况的机制。因此，更好地了解KIR-MHC I类相互作用的分子机制可以促进疾病的治疗策略。目前的审查集中在疾病中各种KIR及其与相关配体的组合的主要特征和功能细节，并着重指出了在基于NK细胞的免疫疗法中操纵关键检查点的持续努力。