The Iowa Cancer Research Foundation, Urbandale, IA 50322, USA.
Mol Immunol. 2010 Nov-Dec;48(1-3):9-13. doi: 10.1016/j.molimm.2010.08.016. Epub 2010 Sep 25.
The complement cascade of the immune system is an important mediator of the inflammatory response to infection; however it is crucial that this pathway is tightly regulated to prevent uncontrolled activation, which can lead to damage to host tissues. The complement system has many regulators that control activation; both membrane-bound and soluble factors. This review will focus on what is currently known about the transcriptional regulation of the soluble complement regulatory genes C1-inhibitor, complement factor I, complement factor H and C4-binding protein. The absence or mutation of these regulators is all associated with specific disease, and yet their contribution to disease is often poorly understood. It is through full understanding of these genes that we can comprehend the diseases with which they are implicated, and thus prove why knowledge of the transcriptional regulation of these genes is valuable.
免疫系统的补体级联反应是感染引起炎症反应的重要介质；然而，至关重要的是，这条途径必须受到严格的调控，以防止不受控制的激活，否则可能导致宿主组织损伤。补体系统有许多调节剂可以控制激活；包括膜结合和可溶性因子。本综述将重点介绍目前对可溶性补体调节基因 C1 抑制剂、补体因子 I、补体因子 H 和 C4 结合蛋白的转录调控的了解。这些调节剂的缺失或突变都与特定疾病相关，但它们对疾病的贡献往往知之甚少。只有充分了解这些基因，我们才能理解它们所涉及的疾病，从而证明了解这些基因的转录调控是有价值的。
