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干燥综合征中B细胞表型、免疫球蛋白基因表达异常及自身免疫的出现。

Abnormalities of B cell phenotype, immunoglobulin gene expression and the emergence of autoimmunity in Sjögren's syndrome.

作者信息

Dörner Thomas, Lipsky Peter E

机构信息

Department of Medicine, Rheumatology and Clinical Immunology, University Hospital Charité, Berlin, Germany.

出版信息

Arthritis Res. 2002;4(6):360-71. doi: 10.1186/ar603. Epub 2002 Sep 25.

Abstract

Primary Sjögren's syndrome (pSS) is an autoimmune disorder characterized by specific pathologic features and the production of typical autoantibodies. In addition, characteristic changes in the distribution of peripheral B cell subsets and differences in use of immunoglobulin variable-region genes are also features of pSS. Comparison of B cells from the blood and parotid gland of patients with pSS with those of normal donors suggests that there is a depletion of memory B cells from the peripheral blood and an accumulation or retention of these antigen-experienced B cells in the parotids. Because disordered selection leads to considerable differences in the B cell repertoire in these patients, the delineation of its nature should provide important further clues to the pathogenesis of this autoimmune inflammatory disorder.

摘要

原发性干燥综合征(pSS)是一种自身免疫性疾病,其特征为特定的病理特征和典型自身抗体的产生。此外,外周B细胞亚群分布的特征性变化以及免疫球蛋白可变区基因使用的差异也是pSS的特征。将pSS患者血液和腮腺中的B细胞与正常供体的B细胞进行比较表明,外周血中记忆B细胞减少,而这些经历过抗原刺激的B细胞在腮腺中积累或滞留。由于无序选择导致这些患者的B细胞库存在显著差异,因此对其性质的描述应为这种自身免疫性炎症性疾病的发病机制提供重要的进一步线索。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f97/153845/113a93b74c99/ar603-1.jpg

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