Protein coats are supramolecular complexes that assemble on the cytosolic face of membranes to promote cargo sorting and transport carrier formation in the endomembrane system of eukaryotic cells. Several types of protein coats have been described, including COPI, COPII, AP-1, AP-2, AP-3, AP-4, AP-5, and retromer, which operate at different stages of the endomembrane system. Defects in these coats impair specific transport pathways, compromising the function and viability of the cells. In humans, mutations in subunits of these coats cause various congenital diseases that are collectively referred to as coatopathies. In this article, we review the fundamental properties of protein coats and the diseases that result from mutation of their constituent subunits.

中文翻译：

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Coatopathies：蛋白外套的遗传疾病。

蛋白质外壳是超分子复合物，其组装在膜的胞质表面上，以促进货物分选和运输真核细胞内膜系统中的载体形成。已经描述了几种类型的蛋白质外壳，包括COPI，COPII，AP-1，AP-2，AP-3，AP-4，AP-5和逆向异构体，它们在膜内系统的不同阶段起作用。这些被毛的缺陷会破坏特定的运输途径，损害细胞的功能和生存能力。在人类中，这些皮毛亚单位的突变会导致多种先天性疾病，这些疾病统称为“涂膜病”。在本文中，我们回顾了蛋白外壳的基本特性以及由其组成亚基突变引起的疾病。