Comprehensive Pneumology Center (CPC), University Hospital, Ludwig Maximilians University, Helmholtz Zentrum München, Germany; Comprehensive Pneumology Center, Munich (CPC-M), Germany; Member of the German Center for Lung Research (DZL), Munich, Germany.
Pulmonary, Allergy, and Critical Care Medicine, Acute Lung Injury Center of Excellence, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA.
Transl Res. 2018 Aug;198:17-28. doi: 10.1016/j.trsl.2018.03.003. Epub 2018 Mar 29.
The present review aims to summarize available knowledge on the role of the ubiquitin-proteasome system (UPS) in the pathogenesis of scleroderma and scleroderma-related disease mechanisms. This will provide the reader with a more mechanistic understanding of disease pathogenesis and help to identify putative novel targets within the UPS for potential therapeutic intervention. Because of the heterogenous manifestations of scleroderma, we will primarily focus on conserved mechanisms that are involved in the development of lung scleroderma phenotypes.
本篇综述旨在总结泛素-蛋白酶体系统 (UPS) 在硬皮病发病机制及硬皮病相关疾病机制中的作用的现有知识。这将使读者对疾病发病机制有更深入的了解,并有助于确定 UPS 中潜在的新治疗靶点。由于硬皮病的表现形式多样,我们将主要关注涉及肺硬皮病表型发生的保守机制。