Muthana Munitta, Hawtree Sarah, Wilshaw Adam, Linehan Eimear, Roberts Hannah, Khetan Sachin, Adeleke Gbadebo, Wright Fiona, Akil Mohammed, Fearon Ursula, Veale Douglas, Ciani Barbara, Wilson Anthony G
Academic Unit of Rheumatology, University of Sheffield, Sheffield S10 2RX, United Kingdom;
Department of Rheumatology, Dublin Academic Medical Centre and the Conway Institute of Biomolecular and Biomedical Research, University College Dublin, Dublin D04 W6F6, Ireland;
Proc Natl Acad Sci U S A. 2015 Sep 15;112(37):11618-23. doi: 10.1073/pnas.1501947112. Epub 2015 Aug 27.
The variant rs26232, in the first intron of the chromosome 5 open reading frame 30 (C5orf30) locus, has recently been associated with both risk of developing rheumatoid arthritis (RA) and severity of tissue damage. The biological activities of human C5orf30 are unknown, and neither the gene nor protein show significant homology to any other characterized human sequences. The C5orf30 gene is present only in vertebrate genomes with a high degree of conservation, implying a central function in these organisms. Here, we report that C5orf30 is highly expressed in the synovium of RA patients compared with control synovial tissue, and that it is predominately expressed by synovial fibroblast (RASF) and macrophages in the lining and sublining layer of the tissue. These cells play a central role in the initiation and perpetuation of RA and are implicated in cartilage destruction. RASFs lacking C5orf30 exhibit increased cell migration and invasion in vitro, and gene profiling following C5orf30 inhibition confirmed up-regulation of genes involved in cell migration, adhesion, angiogenesis, and immune and inflammatory pathways. Importantly, loss of C5orf30 contributes to the pathology of inflammatory arthritis in vivo, because inhibition of C5orf30 in the collagen-induced arthritis model markedly accentuated joint inflammation and tissue damage. Our study reveal C5orf30 to be a previously unidentified negative regulator of tissue damage in RA, and this protein may act by modulating the autoaggressive phenotype that is characteristic of RASFs.
位于5号染色体开放阅读框30(C5orf30)基因座第一内含子中的rs26232变异体,最近被发现与类风湿关节炎(RA)的发病风险以及组织损伤的严重程度均相关。人类C5orf30的生物学活性尚不清楚,该基因和蛋白质与其他任何已明确的人类序列均无显著同源性。C5orf30基因仅存在于脊椎动物基因组中,且具有高度保守性,这意味着它在这些生物体中具有核心功能。在此,我们报告,与对照滑膜组织相比,C5orf30在RA患者的滑膜中高表达,且在组织衬里层和衬里下层的滑膜成纤维细胞(RASF)和巨噬细胞中占主导表达。这些细胞在RA的起始和持续过程中起核心作用,并与软骨破坏有关。缺乏C5orf30的RASF在体外表现出细胞迁移和侵袭增加,C5orf30抑制后的基因谱分析证实了参与细胞迁移、黏附、血管生成以及免疫和炎症途径的基因上调。重要的是,C5orf30的缺失在体内促成了炎性关节炎的病理过程,因为在胶原诱导的关节炎模型中抑制C5orf30可显著加重关节炎症和组织损伤。我们的研究揭示C5orf30是RA中一种先前未被识别的组织损伤负调节因子,该蛋白可能通过调节RASF特有的自身攻击表型发挥作用。