Saito Megumu K
Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University, Kyoto 6068507, Japan.
Children (Basel). 2021 Feb 1;8(2):94. doi: 10.3390/children8020094.
Autoinflammatory diseases are a disease entity caused by the dysregulation of innate immune cells. Typical autoinflammatory diseases are monogenic disorders and often very rare. As a result, there is a relative lack of understanding of the pathogenesis, poor diagnosis and little available treatment. Induced pluripotent stem (iPS) cells are a new technology being applied to in vitro disease modeling. These models are especially useful for the analysis of rare and intractable diseases including autoinflammatory diseases. In this review, I will provide a general overview of iPS cell models for autoinflammatory diseases and a brief description of the results obtained from individual reports.
自身炎症性疾病是一种由固有免疫细胞失调引起的疾病实体。典型的自身炎症性疾病是单基因疾病，通常非常罕见。因此，人们对其发病机制相对缺乏了解，诊断困难，可用的治疗方法也很少。诱导多能干细胞（iPS细胞）是一种应用于体外疾病建模的新技术。这些模型对于分析包括自身炎症性疾病在内的罕见和难治性疾病特别有用。在这篇综述中，我将概述用于自身炎症性疾病的iPS细胞模型，并简要描述从个别报告中获得的结果。
