Hedgehog信号通路在系统性硬皮病发病机制中的作用  

The Pathogenesis of Hedgehog Signaling Pathway in Systemic Scleroderma

作　　者：黄敏 迟素敏 李承新 HUANG Min;CHI Sumin;LI Chengxin(Department of Dermatology,the First Medical Center,Chinese PLA General Hospital,Beijing 100853,China;Department of Physiology,Basic Medical University of Air Force,Xi'an 710032,China)

机构地区：[1]中国人民解放军总医院第一医学中心皮肤科,北京100853 [2]空军军医大学基础部生理教研室,陕西西安710032

出　　处：《中国皮肤性病学杂志》2023年第4期480-483,共4页The Chinese Journal of Dermatovenereology

基　　金：国家自然科学基金项目(81972936,81673043)。

摘　　要：系统性硬皮病(systemic sclerosis, SSc)是一种典型的纤维化疾病,可累及皮肤、心脏、肺、胃肠道等多个器官。SSc的发病机制尚未完全阐明,目前认为SSc发病的主要机制是成纤维细胞异常活化,分泌过多的细胞外基质所致,但成纤维细胞持续活化的机制仍不明确。已有研究表明多种信号通路参与了成纤维细胞活化,如Hedgehog(Hh)、Wnt/β-catenin、Notch、TGF-β等。本文主要针对Hh信号通路及其在SSc发病机制中的作用进行综述,探讨系统性硬皮病纤维化的可能发生机制及潜在的治疗靶点。Systemic sclerosis(SSc)is a typical fibrotic disease that affects the skin and a variety of internal organs,including heart,lung,gastrointestinal tract and other organs.However,the pathogenesis of SSc has been partially identified.The main mechanism of SSc is due to the abnormal activation of fibroblasts,which secrete excessive extracellular matrix(ECM).However,the mechanism about persistent activation of fibroblasts is incompletely understood.It has been shown that many signaling pathways are involved in the activation of fibroblasts,such as Hedgehog(Hh),Wnt/β-Catenin,Notch,TGF-β.This review is focused on the relationship between Hh signaling pathway and systemic scleroderma,the possible mechanism and potential therapeutic target of Hh signaling pathway in systemic scleroderma fibrosis.

关 键 词：HEDGEHOG信号通路 系统性硬皮病 发病机制 

分 类 号：R593.25[医药卫生—内科学]