The NZB/W F1 (F1) mice develop severe disease that is similar to human systemic lupus erythematosus. By contrast, each parent strain, NZB or NZW, has limited autoimmunity, suggesting traits of both strains contribute to pathogenesis. Although many of the contributing genes have been identified, the contributing cellular abnormality associated with each parent strain remains unresolved. Given that plasmacytoid dendritic cells (pDCs) are key to the pathogenesis of lupus, we investigated the properties of pDCs from NZB and NZW mice. We found that NZB mouse had higher numbers of pDCs, with much of the increase being contributed by a more abundant CD8+ pDC subset. This was associated with prolonged survival and stronger proliferation of CD4+ T cells. By contrast, NZW pDCs had heightened capacity to produce interferon-α (IFNα) and IFNλ, and promoted stronger B-cell proliferation upon CpG stimulation. Thus, our data reveal the different functional and numerical characteristics of pDCs from NZW and NZB mouse.

中文翻译：

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NZB / W F1狼疮小鼠亲本株的浆细胞样树突状细胞对自身免疫倾向具有不同的特征。

NZB / W F1（F1）小鼠患上严重的疾病，类似于人系统性红斑狼疮。相比之下，每种亲本菌株NZB或NZW的自身免疫力均有限，这表明这两种菌株的性状均有助于发病。尽管已经鉴定了许多贡献基因，但与每个亲本菌株相关的贡献细胞异常仍未解决。鉴于浆细胞样树突状细胞（pDCs）是狼疮发病的关键，我们研究了NZB和NZW小鼠的pDCs的特性。我们发现NZB小鼠的pDC数量更多，其中大部分增加是由更丰富的CD8 + pDC亚群引起的。这与CD4 + T细胞的延长生存期和更强的增殖有关。相比之下，NZW pDC具有更高的产生干扰素-α（IFNα）和IFNλ的能力，并在CpG刺激下促进更强的B细胞增殖。因此，我们的数据揭示了来自NZW和NZB小鼠的pDC的不同功能和数值特征。