Clinical associations and expression pattern of the autoimmunity susceptibility factor DIORA-1 in patients with primary Sjögren’s syndrome

医学 自身免疫 免疫学 类风湿因子 关节炎 抗体
作者
Lara A. Aqrawi,Lara Mentlein,Lauro Meneghel,Albin Björk,Guðný Ella Thorlacius,Margarita Ivanchenko,Jorge I. Ramírez Sepúlveda,Kathrine Skarstein,Marika Kvarnström,Susanna Brauner,Alexander Espinosa,Marie Wahren‐Herlenius
出处
期刊:Annals of the Rheumatic Diseases [BMJ]
卷期号:77 (12): 1840-1842 被引量:5
标识
DOI:10.1136/annrheumdis-2018-213634
摘要

Sjogren’s syndrome (SS) is characterised by B cell abnormalities and immune-mediated destruction of exocrine glands, primarily the salivary and lacrimal glands.1 2 Among the reported genetic polymorphisms associated with primary SS (pSS), the FAM167A-BLK locus distinguishes itself as an interesting candidate for further analysis based on the strong expression quantitative locus effect of pSS-associated polymorphisms on FAM167A (member A of the Family with sequence similarity 167), contrasted with only moderate or no effect on BLK .3 4 Little is known about the FAM167A gene and its relevance to rheumatic disease pathogenesis. We recently explored FAM167A and its encoded protein Disordered autoimmunity-1 (DIORA-1),4 and reported that DIORA-1 is conserved in vertebrates, has an intracellular, cytoplasmic localisation and in mice is predominantly expressed in lung and spleen—two organs with a high content of immune cells. In the present study, we investigated the expression of DIORA-1 in human immune cells and in salivary glands of patients with pSS, and assessed DIORA-1 expression in relation to pSS clinical manifestations. Notably, we observed expression of DIORA-1 in CD19+ B cells, but little or no expression in monocytes or T cells (figure 1A). DIORA-1 expression in CD19+ B cells was similar in patients with pSS and healthy donors (figure 1B). To further define the expression pattern in B cells, we analysed DIORA-1 expression in cell lines representing discrete …
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