板层小体与相关皮肤病研究进展

Update of the lamellar bodies and related dermatosis

板层小体是一种反面高尔基体或溶酶体相关的分泌性细胞器,其合成和分泌受高尔基pH调节器、鱼鳞病基因、细胞外环境等多因素调控。表皮内板层小体包含如脂类、酶类、蛋白酶抑制剂、抗微生物肽等装载物,是参与形成皮肤多种屏障的重要结构。表皮内板层小体异常与一些皮肤屏障受损皮肤病的病理机制相关,如特应性皮炎、鱼鳞病相关综合征等。本文对板层小体与相关皮肤病研究进展进行综述。

The lamellar bodies are secretory organelles derived from the cisternae of trans-Golgi network or lysosome.The process of synthesis and secretion is regulated by Golgi pH regulator,genes involved in ichthyosis and the extracellular environment,ect.The epidermal lamellar bodies contain many cargoes,such as lipids,enzymes,protease inhibitors and antimicrobial peptides,et al.play important roles in the formation of skin barriers.A variety of skin diseases are associated with the epidermal lamellar bodies abnormalities,such as atopic dermatitis and ichthyosis syndrome.The update of the lamellar bodies and related dermatosis is reviewed in this paper.