Center of Medical Genetics, University of Antwerp and Antwerp University Hospital, Antwerp, Belgium.
Department of Clinical Genetics, Radboud University Medical Center, Nijmegen, The Netherlands.
Am J Physiol Cell Physiol. 2022 Jun 1;322(6):C1214-C1222. doi: 10.1152/ajpcell.00036.2022. Epub 2022 Apr 27.
The class I small leucine-rich proteoglycan biglycan is a crucial structural extracellular matrix component that interacts with a wide range of extracellular matrix molecules. In addition, biglycan is involved in sequestering growth factors such as transforming growth factor-β and bone morphogenetic proteins and thereby regulating pathway activity. Biglycan consists of a 42-kDa core protein linked to two glycosaminoglycan side chains and both are involved in protein interactions. Biglycan is encoded by the gene located on the X-chromosome and is expressed in various tissues, including vascular tissue, skin, brain, kidney, lung, the immune system, and the musculoskeletal system. Although an increasing amount of data on the biological function of biglycan in the vasculature has been produced, its role in thoracic aortic aneurysms is still not fully elucidated. This review focuses on the role of biglycan in the healthy thoracic aorta and the development of thoracic aortic aneurysm and dissections in both mice and humans.
I 型小富含亮氨酸的蛋白聚糖 biglycan 是细胞外基质的重要结构成分，可与多种细胞外基质分子相互作用。此外，biglycan 还参与了生长因子如转化生长因子-β和骨形态发生蛋白的隔离，从而调节通路活性。Biglycan 由一个 42kDa 的核心蛋白与两个糖胺聚糖侧链相连，两者都参与了蛋白相互作用。Biglycan 由位于 X 染色体上的 基因编码，在多种组织中表达，包括血管组织、皮肤、大脑、肾脏、肺、免疫系统和骨骼肌肉系统。尽管越来越多的关于 biglycan 在血管中的生物学功能的数据已经产生，但它在胸主动脉瘤中的作用仍未完全阐明。这篇综述重点介绍了 biglycan 在健康的胸主动脉以及胸主动脉瘤和夹层在小鼠和人类中的发展中的作用。
