MULTIPLE DEFECTS IN THE IMMUNE-SYSTEM OF LYN-DEFICIENT MICE, CULMINATING IN AUTOIMMUNE-DISEASE
MULTIPLE DEFECTS IN THE IMMUNE-SYSTEM OF LYN-DEFICIENT MICE, CULMINATING IN AUTOIMMUNE-DISEASE
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DOI：
10.1016/0092-8674(95)90171-x
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发表时间：
1995-10-20
期刊：
影响因子：
64.5
通讯作者：
DUNN, ARR
中科院分区：
文献类型：
--
作者：
HIBBS, ML;TARLINTON, DM;DUNN, ARR
在林恩基因座处的破坏的纯合子小鼠显示与B淋巴细胞谱系和肥大细胞功能相关的异常。尽管再循环B淋巴细胞的数量减少，林恩（-/-）小鼠是免疫球蛋白M（IgM）高球蛋白血症。对T非依赖性和T依赖性抗原的免疫应答受到影响。林恩（-/-）小鼠不能介导对IgE交联的过敏反应，表明林恩的活化在Fc γ RI信号传导中起不可或缺的作用。林恩（-/-）小鼠具有循环自身反应性抗体，并且许多小鼠显示由IgG免疫复合物在肾脏中沉积引起的严重肾小球肾炎，这是一种使人联想到系统性红斑狼疮的病理学。总的来说，这些结果暗示林恩在免疫球蛋白介导的信号传导中具有不可或缺的作用，特别是在建立B细胞耐受性中。
Mice homozygous for a disruption at the Lyn locus display abnormalities associated with the B lymphocyte lineage and in mast cell function, Despite reduced numbers of recirculating B lymphocytes, Lyn(-/-) mice are immunoglobulin M (IgM) hyperglobulinemic. Immune responses to T-independent and T-dependent antigens are affected. Lyn(-/-) mice fail to mediate an allergic response to IgE cross-linking, indicating that activation of LYN plays an indispensable role in Fc epsilon RI signaling. Lyn(-/-) mice have circulating autoreactive antibodies, and many show severe glomerulonephritis caused by the deposition of IgG immune complexes in the kidney, a pathology reminiscent of systemic lupus erythematosus. Collectively, these results implicate LYN as having an indispensable role in immunoglobulin-mediated signaling, particularly in establishing B cell tolerance.