Dillinger Andrea E, Hoffmann Anja K, Tamm Ernst R
Institute of Human Anatomy and Embryology, University of Regensburg, Regensburg, Germany.
Institute of Neuroanatomy, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Adv Exp Med Biol. 2025;1468:423-427. doi: 10.1007/978-3-031-76550-6_69.
Chondroitin sulfate proteoglycans are characterized by their sulfated glycosaminoglycan chains covalently attached to the core protein. Versican, encoded by the CSPG2 gene, is a chondroitin sulfate proteoglycan highly expressed in the human and mouse retina. Due to alternative splicing, it exists in four different isoforms: V0, V1, V2 and V3. Interactions with a variety of proteins lead to its main functions, such as influencing cellular adhesion, migration, proliferation and regulating inflammatory processes. Versican expression is high during retinal development and decreases in the mature tissue, remaining mostly in the retinal pigment epithelium, Bruch's membrane and choroid blood vessel walls. Mutations in the CSPG2 gene resulting in an imbalance of Versican splice variants cause erosive vitreoretinopathy and Wagner disease. Its regulatory role in a variety of functions, especially those influencing extracellular matrix composition and inflammation, suggests a crucial role in the pathomechanisms of retinal degenerative disorders and should be the purpose of future research.
硫酸软骨素蛋白聚糖的特征在于其硫酸化的糖胺聚糖链共价连接至核心蛋白。由CSPG2基因编码的多功能蛋白聚糖是一种在人和小鼠视网膜中高度表达的硫酸软骨素蛋白聚糖。由于可变剪接,它以四种不同的异构体形式存在:V0、V1、V2和V3。与多种蛋白质的相互作用导致其主要功能,例如影响细胞粘附、迁移、增殖以及调节炎症过程。多功能蛋白聚糖在视网膜发育过程中表达较高,而在成熟组织中表达降低,主要保留在视网膜色素上皮、布鲁赫膜和脉络膜血管壁中。CSPG2基因的突变导致多功能蛋白聚糖剪接变体失衡,从而引起侵蚀性玻璃体视网膜病变和瓦格纳病。它在多种功能中的调节作用,尤其是那些影响细胞外基质组成和炎症的功能,表明其在视网膜退行性疾病的发病机制中起关键作用,应成为未来研究的目标。