利用汉族斑驳病大家系研究人DC c-kit信号通路对Th细胞分化的影响

)：.Th 细胞分化直接影响免疫应答的类型。动物研究证实c-Kit信号通路在树突状细胞（DC）调控Th 细胞分化中起关键作用。该发现提示可以通过控制DC c-Kit表达来改变免疫状态从而达到治疗免疫相关疾病的目的。但基于实验动物的研究与人体情况存在明显差别，DC c-kit信号通路的免疫调控功能值得进一步研究。近期，我们鉴定的一个汉族斑驳病大家系为开展此研究提供了机遇。前期工作已查明该家系致病位点为一新突变（1900insATGA），家系成员众多且皮损轻重程度各异。本课题拟以该家系为研究对象，采用免疫学方法和高通量基因芯片技术对人DC 中c-Kit 调控Th细胞分化的一系列关键事件进行定性和定量研究，以期证实c-Kit 信号通路在人DC 调控Th 细胞分化中起关键作用,为研究新的DC 生物治疗策略提供理论基础和实验依据。

Th cell differentiation directly affect the type of immune response. Animal studies have confirmed the c-Kit signaling pathway plays a key role in dendritic cells (DC) regulation of Th cell differentiation. The findings suggest that the DC of c-Kit expression to change the immune status so as to achieve the purpose of the treatment of immune-related diseases, may have significant clinical value. There are obvious differences between based on experimental animal studies and human DC。c-kit signaling pathway in immune regulation function deserves further study. Recently, we identified a Han piebaldism pedigree to carry out this study opportunities. Preliminary work to identify pathogenicity locus of the family to a new mutation (1900insATGA), many family members and lesions severity vary. This topic is intended to be the pedigree object of study, a series of key events in the regulation of Th cell differentiation of mature immunological methods and high-throughput gene chip technology on human DC in c-Kit for qualitative and quantitative research, in order to confirm that c-Kit play a key role in human DC regulation of Th cell differentiation and provide a theoretical basis and experimental basis for the study of new DC biological treatment strategies.