斑蛋白的病生理功能  

Pathophysiological Functions of Plakins

作　　者：王瑞[1,2,3] 陈喜雪 朱学骏[1,2,3] 王明悦 WANG Rui;CHEN Xixue;ZHU Xuejun;WANG Mingyue(Department of Dermatology,Peking University First Hospital,Beijing 100034,China;National Clinical Research Center for Skin and Immune Diseases,Beijing 100034,China;Beijing Key Laboratory of Molecular Diagnosis on Dermatoses,Beijing 100034,China)

机构地区：[1]北京大学第一医院皮肤性病科,北京100000 [2]国家皮肤与免疫疾病临床医学研究中心,北京100000 [3]皮肤病分子诊断北京市重点实验室,北京100000

出　　处：《皮肤科学通报》2021年第3期259-262,共4页Dermatology Bulletin

基　　金：国家自然科学基金重点项目(81130030);国家自然科学基金青年项目(81000694);北京市自然科学基金面上项目(7172214)共同资助。

摘　　要：斑蛋白家族是一组同源性较高的大分子量结构蛋白,这些蛋白在皮肤、神经、肌肉、血管等多种组织中均有表达,起到交联构成细胞骨架和黏附结构如桥粒、半桥粒的作用。斑蛋白的一些先天性缺陷和后天的免疫损伤可导致一系列的疾病。因此,对于这一类重要蛋白的深入研究,有助于我们从共同的病因角度认识这一组看似并不相关的疾病。Plakin family is a group of large structural proteins with high homology.These proteins are expressed in the skin,nerve,muscle,blood vessel and other tissues,and play the role of cross-linking to form cytoskeleton and adhesion structure such as desmosomes and semidesmosomes.Some congenital defects of plakin proteins and acquired immune damage can lead to a series of diseases.Therefore,deep investigation of these important proteins will help us to understand this group of seemingly unrelated diseases from the perspective of common etiology.

关 键 词：斑蛋白 桥斑蛋白 包斑蛋白 周斑蛋白 副肿瘤性天疱疮 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]