Pugh-Bernard Aimee E, Cambier John C
Integrated Department of Immunology, University of Colorado Health Sciences Center and National Jewish Medical Research Center, 1400 Jackson Street, Denver, CO 80206, USA.
Curr Opin Rheumatol. 2006 Sep;18(5):451-5. doi: 10.1097/01.bor.0000240353.99808.5f.
The purpose of this review is to inform the scientific community of the most recent findings surrounding B cell receptor signaling function in human systemic lupus erythematosus and how altered B cell signaling may explain the characteristic hyperactivity of B cells in active disease and contribute to its pathogenesis.
B cell receptor signaling is abnormal in patients with active systemic lupus erythematosus as demonstrated by increased calcium flux and global B cell hyperactivity. Altered signaling has been explained by a variety of factors such as defective FcgammaRIIB signaling, decreased expression of the protein tyrosine kinase Lyn, and increased serum levels of B lymphocyte stimulator.
The studies reviewed suggest that B cells from systemic lupus erythematosus patients display molecular signaling defects that most likely contribute to pathogenesis of the disease and explain the characteristic hyperactivity of B cells in active disease.
本综述旨在向科学界通报有关人类系统性红斑狼疮中B细胞受体信号传导功能的最新研究结果，以及B细胞信号改变如何解释活动性疾病中B细胞的特征性高活性并促进其发病机制。
活动性系统性红斑狼疮患者的B细胞受体信号异常，表现为钙流增加和整体B细胞高活性。信号改变已由多种因素解释，如FcγRIIB信号缺陷、蛋白酪氨酸激酶Lyn表达降低以及B淋巴细胞刺激因子血清水平升高。
所综述的研究表明，系统性红斑狼疮患者的B细胞存在分子信号缺陷，这很可能导致疾病的发病机制，并解释了活动性疾病中B细胞的特征性高活性。
