Department of Physiology, David Geffen School of Medicine at UCLA, Los Angeles, California, USA.
Int Rev Cell Mol Biol. 2011;288:1-41. doi: 10.1016/B978-0-12-386041-5.00001-7.
Vitamin A is essential for diverse aspects of life ranging from embryogenesis to the proper functioning of most adult organs. Its derivatives (retinoids) have potent biological activities such as regulating cell growth and differentiation. Plasma retinol-binding protein (RBP) is the specific vitamin A carrier protein in the blood that binds to vitamin A with high affinity and delivers it to target organs. A large amount of evidence has accumulated over the past decades supporting the existence of a cell-surface receptor for RBP that mediates cellular vitamin A uptake. Using an unbiased strategy, this specific cell-surface RBP receptor has been identified as STRA6, a multitransmembrane domain protein with previously unknown function. STRA6 is not homologous to any protein of known function and represents a new type of cell-surface receptor. Consistent with the diverse functions of vitamin A, STRA6 is widely expressed in embryonic development and in adult organ systems. Mutations in human STRA6 are associated with severe pathological phenotypes in many organs such as the eye, brain, heart, and lung. STRA6 binds to RBP with high affinity and mediates vitamin A uptake into cells. This review summarizes the history of the RBP receptor research, its expression in the context of known functions of vitamin A in distinct human organs, structure/function analysis of this new type of membrane receptor, pertinent questions regarding its very existence, and its potential implication in treating human diseases.
维生素 A 是生命中从胚胎发生到大多数成年器官正常功能所必需的。其衍生物(类视黄醇)具有调节细胞生长和分化等强大的生物学活性。血浆视黄醇结合蛋白(RBP)是血液中特定的维生素 A 载体蛋白,它与维生素 A 具有高亲和力,并将其递送到靶器官。过去几十年积累了大量证据支持 RBP 存在介导细胞维生素 A 摄取的细胞表面受体。使用一种无偏见的策略,已经确定这种特定的细胞表面 RBP 受体是 STRA6,一种具有先前未知功能的多跨膜域蛋白。STRA6 与任何已知功能的蛋白质都没有同源性,代表了一种新型的细胞表面受体。与维生素 A 的多种功能一致,STRA6 在胚胎发育和成人器官系统中广泛表达。人类 STRA6 的突变与许多器官(如眼睛、大脑、心脏和肺)的严重病理表型有关。STRA6 与 RBP 具有高亲和力,并介导维生素 A 进入细胞摄取。这篇综述总结了 RBP 受体研究的历史,它在不同人类器官中维生素 A 已知功能方面的表达,这种新型膜受体的结构/功能分析,关于其存在的相关问题,以及它在治疗人类疾病方面的潜在意义。