Sofia University "St. Kliment Ohridski", Faculty of Biology, 8 Dragan Tzankov str., 1164 Sofia, Bulgaria.
Sofia University "St. Kliment Ohridski", Faculty of Biology, 8 Dragan Tzankov str., 1164 Sofia, Bulgaria.
Adv Colloid Interface Sci. 2022 Apr;302:102619. doi: 10.1016/j.cis.2022.102619. Epub 2022 Feb 22.
The transmembrane Ca - activated Cl channel - human bestrophin-1 (hBest1) is expressed in retinal pigment epithelium and mutations of BEST1 gene cause ocular degenerative diseases colectivelly referred to as "bestrophinopathies". A large number of genetical, biochemical, biophysical and molecular biological studies have been performed to understand the relationship between structure and function of the hBest1 protein and its pathophysiological significance. Here, we review the current understanding of hBest1 surface organization, interactions with membrane lipids in model membranes, and its association with microdomains of cellular membranes. These highlights are significant for modulation of channel activity in cells.
跨膜 Ca2+激活氯离子通道——人源 bestrophin-1(hBest1)在视网膜色素上皮细胞中表达,BEST1 基因突变会导致眼部退行性疾病,通常被称为“bestrophinopathy”。为了了解 hBest1 蛋白的结构与功能关系及其病理生理学意义,进行了大量的遗传学、生物化学、生物物理学和分子生物学研究。本文综述了 hBest1 表面结构、在模型膜中与膜脂相互作用及其与细胞膜微区室的关联等方面的最新研究进展。这些研究结果对调节细胞内通道活性具有重要意义。