Saito Megumu K
a Department of Clinical Application, CiRA , Kyoto University , Kyoto , Japan.
Immunol Med. 2018 Jun;41(2):68-74. doi: 10.1080/13497413.2018.1481583. Epub 2018 Sep 7.
Disease-associated induced pluripotent stem cells (iPSCs) established from patients are now widely used for disease modeling. They can provide an unlimited source of hematopoietic cells that carry the patients' genetic background, making them advantageous for modeling immunological disorders. To obtain functional immune cells from human iPSCs, we have developed a differentiation system that generates immortalized myeloid cells including neutrophils and monocytic cells. By using this strategy, we have established in vitro models of many immunological disorders. In this review, we focus on autoinflammatory disorders. These models have proven useful for genetic diagnosis and elucidation of the disease mechanism.
从患者身上建立的疾病相关诱导多能干细胞（iPSC）目前被广泛用于疾病建模。它们可以提供携带患者遗传背景的无限造血细胞来源，使其在免疫紊乱建模方面具有优势。为了从人iPSC中获得功能性免疫细胞，我们开发了一种分化系统，可生成包括中性粒细胞和单核细胞在内的永生化髓系细胞。通过使用这种策略，我们建立了许多免疫紊乱的体外模型。在这篇综述中，我们重点关注自身炎症性疾病。这些模型已被证明对基因诊断和疾病机制的阐明有用。
