Division of Bone and Mineral Research, Department of Medicine, Washington University School of Medicine, St. Louis, MO 63110, United States.
Department of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, MO 63110, United States.
Matrix Biol. 2018 Oct;71-72:100-111. doi: 10.1016/j.matbio.2018.03.006. Epub 2018 Mar 7.
Microfibril-associated glycoproteins 1 and 2 (MAGP-1, MAGP-2) are protein components of extracellular matrix microfibrils. These proteins interact with fibrillin, the core component of microfibrils, and impart unique biological properties that influence microfibril function in vertebrates. MAGPs bind active forms of TGFβ and BMPs and are capable of modulating Notch signaling. Mutations in MAGP-1 or MAGP-2 have been linked to thoracic aneurysms and metabolic disease in humans. MAGP-2 has also been shown to be an important biomarker in several human cancers. Mice lacking MAGP-1 or MAGP-2 have defects in multiple organ systems, which reflects the widespread distribution of microfibrils in vertebrate tissues. This review summarizes our current understanding of the function of the MAGPs and their relationship to human disease.
微纤维相关糖蛋白 1 和 2(MAGP-1、MAGP-2)是细胞外基质微纤维的蛋白成分。这些蛋白与微纤维的核心成分原纤维蛋白相互作用,并赋予其独特的生物学特性,影响脊椎动物的微纤维功能。MAGPs 结合 TGFβ和 BMP 的活性形式,并能够调节 Notch 信号。MAGP-1 或 MAGP-2 的突变与人类的胸主动脉瘤和代谢疾病有关。MAGP-2 也被证明是几种人类癌症的重要生物标志物。缺乏 MAGP-1 或 MAGP-2 的小鼠在多个器官系统中存在缺陷,这反映了微纤维在脊椎动物组织中的广泛分布。本文综述了我们目前对 MAGPs 功能及其与人类疾病关系的认识。