Abstract:

Human SP140， a leukocyte-specific nuclear protein inhibited by GSK761， belongs to the speck-like protein （SP） family and is predominantly expressed in immune cells. Current research indicates that SP140 activates gene transcription， regulates gene expression， and plays a significant role in immune modulation. This review systematically summarized the fundamental molecular characteristics of SP140， its role in the regulation of gene expression， and its involvement in immune responses. Furthermore， it detailed the association of SP140 with numerous diseases， primarily immune-related disorders， including primary biliary cholangitis （formerly cirrhosis）， multiple sclerosis， Crohn's disease， chronic lymphocytic leukemia， acquired immune deficiency syndrome（AIDS）， in order to enhance the understanding of the pathogenesis of these diseases and potentially contributing to improvements in their diagnosis and treatment.

Key words: SP140, gene expression, markers, immune response, disease

摘要：

人SP140是一种白细胞特异性核蛋白，其抑制剂为GSK761。它属于斑点蛋白（speck-like protein，SP）家族，优先在免疫细胞中表达。目前研究发现，SP140可以激活基因转录，参与基因调控，并且在免疫调节中发挥着重要作用。系统综述了SP140的基本分子生物学特征、SP140在基因表达调控方面的作用、SP140与免疫反应的关系。总结了SP140与免疫性疾病的关系，包括原发性胆汁性肝硬化、多发性硬化症、克罗恩病、慢性淋巴细胞白血病、艾滋病等，以期增进对这些疾病发病机理的理解，并有望助力其诊断或治疗的改进。

关键词: SP140, 基因表达, 标志物, 免疫反应, 疾病