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Deficiency of Act1, a critical modulator of B cell function, leads to development of Sjogren's syndrome

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成果类型:
期刊论文
作者:
Qian, Youcun;Giltiay, Natalia;Xiao, Jianhua;Wang, Yue;Tian, Jun;...
通讯作者:
Li, Xiaoxia
作者机构:
[Jorgensen, Trine N.; Xiao, Jianhua; Li, Xiaoxia; Giltiay, Natalia; Qian, Youcun] Cleveland Clin, Dept Immunol, Cleveland, OH 44915 USA.
[Qian, Youcun] Chinese Acad Sci, Shanghai Inst Biol Sci, Inst Hlth Sci, Shanghai, Peoples R China.
[Qian, Youcun] Shanghai Jiao Tong Univ, Sch Med, Shanghai 200030, Peoples R China.
[Xiao, Jianhua] Univ S China, Dept Pathogen Biol, Hengyang, Hunan, Peoples R China.
[Carter, Robert; Wang, Yue] Univ Alabama, Dept Med & Microbiol, Birmingham, AL USA.
通讯机构:
[Li, Xiaoxia] C
Cleveland Clin, Dept Immunol, 9500 Euclid Ave, Cleveland, OH 44915 USA.
语种:
英文
关键词:
Act1;Autoimmunity;B cells;Sjögren's syndrome;Systemic lupus erythematosus
关键词(中文):
动物;抗体;抗核;抗原;自身抗原;B淋巴细胞;肾小球肾炎;小鼠;近交BALB C
期刊:
European Journal of Immunology
ISSN:
0014-2980
年:
2008
卷:
38
期:
8
页码:
2219-2228
基金类别:
Sjögren's Syndrome Foundation Leukemia and Lymphoma Society National Institutes of Health
机构署名:
本校为其他机构
院系归属:
医学院
摘要:
CD40L and B lymphocyte-activating factor (BAFF), members of the TNF superfamily, play critical roles in B cell survival and activation, and in the regulation of humoral immunity. We previously reported that the adaptor molecule Act1 functions as a negative regulator of CD40- and BAFF-mediated B cell survival. Here we demonstrated that mice deficient in Act1 developed systemic autoimmune disease with histological and serological features of human Sjogren's syndrome (SS), in association with systemic lupus erythematosus-like nephritis. Analyses of Act1(-/-)CD40(-/-) and Act1(-/-)BAFF(-/-) double...

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