桥粒蛋白与皮肤病

桥粒位于所有上皮细胞及某些特定组织中。它主要由两类蛋白质构成：跨膜蛋白和胞质内蛋白。某些桥粒蛋白的结构破坏会在不同程度上引起皮肤疾病。这些皮肤疾病主要为自身免疫性皮肤病,如天疱疮、遗传性皮肤病及金黄色葡萄球菌性烫伤样皮肤综合征。主要概述桥粒蛋白的分子结构及其异常所致主要皮肤疾病的发病机制。

Desmosomal proteins and skin diseases

Desmosomes are found in all epithelial cells and some specific tissues. They are mainly composed of two classes of proteins, i.e., transmembrane proteins and intracytoplasmic proteins. The structural destruction of some kinds of desmosomal proteins can lead to skin diseases of different severity. It has been demonstrated that desmosomes are predominantly associated with autoimmune skin diseases such as pemphigus, genodermatosis conditions and staphylococcal scalded skin syndrome. This article outlines the molecular structures of desmosomal proteins as well as the pathogenesis of skin diseases caused by the abnormalities of desmosomal proteins.