系统性硬化症动物模型:最新进展。

IF 5.2 2区 医学 Q1 RHEUMATOLOGY
Current opinion in rheumatology Pub Date : 2023-11-01 Epub Date: 2023-08-21 DOI:10.1097/BOR.0000000000000967
Xiongjie Bi, Tingting Mills, Minghua Wu
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引用次数: 0

摘要

综述目的:系统性硬化症(SSc)是一种多系统自身免疫性结缔组织疾病,其特征是早期炎症,随后皮肤和内脏过度纤维化。加强我们对SSc发病机制的理解对于制定有效的治疗策略至关重要。模拟SSc一个或多个方面的动物模型已被证明是研究疾病机制的宝贵资源。这篇综述旨在提供现有SSc动物模型的最新综述,以及SSc发病机制的潜在相关途径。最近的发现:这篇综述集中在最近生成和研究的动物模型上,这些模型深入研究了现有模型之外的新途径,或利用遗传技术来更深入地了解SSc的发病机制,包括早期I型干扰素(IFN)信号通路的激活,免疫细胞功能和肺动脉高压(PAH)。总结:虽然没有一个单一的动物模型可以完全复制SSc,但不同模型的组合可以为SSc的发病和发展提供有价值的见解。这些见解可以证明动物模型是开发SSc有效治疗方法的关键临床前工具。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Animal models in systemic sclerosis: an update.

Purpose of review: Systemic sclerosis (SSc) is a multisystem autoimmune connective tissue disease characterized by early inflammation followed by excessive fibrosis in the skin and internal organs. Enhancing our comprehension of SSc pathogenesis is essential to develop effective therapeutic strategies. Animal models that mimic one or more aspects of SSc have been proven to be a valuable resource for investigating disease mechanisms. This review aims to provide an updated overview of the existing SSc animal models and the potentially relevant pathways to SSc pathogenesis.

Recent findings: This review focuses on the most recently generated and investigated animal models, which delve into novel pathways beyond existing models or employ genetic technologies to gain a deeper understanding of SSc pathogenesis including activation of early type I interferon (IFN) signaling pathway, immune cell function and pulmonary artery hypertension (PAH).

Summary: While no single animal model can fully replicate SSc, a combination of different models can offer valuable insights into the pathways involved in the onset and advancement of the SSc. These insights can prove animal models as a crutial preclinical tool for developing effective treatments for SSc.

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来源期刊
Current opinion in rheumatology
Current opinion in rheumatology 医学-风湿病学
CiteScore
9.70
自引率
2.00%
发文量
89
审稿时长
6-12 weeks
期刊介绍: A high impact review journal which boasts an international readership, Current Opinion in Rheumatology offers a broad-based perspective on the most recent and exciting developments within the field of rheumatology. Published bimonthly, each issue features insightful editorials and high quality invited reviews covering two or three key disciplines which include vasculitis syndromes, medical physiology and rheumatic diseases, crystal deposition diseases and rheumatoid arthritis. Each discipline introduces world renowned guest editors to ensure the journal is at the forefront of knowledge development and delivers balanced, expert assessments of advances from the previous year.
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