Parfitt David A, Cheetham Michael E
Ocular Biology and Therapeutics, UCL Institute of Ophthalmology, 11-43 Bath Street, EC1V 9EL, London, UK.
Adv Exp Med Biol. 2016;854:479-84. doi: 10.1007/978-3-319-17121-0_64.
Mutations in rhodopsin are one of the most common causes of retinitis pigmentosa (RP). Misfolding of rhodopsin can result in disruptions in cellular protein homeostasis, or proteostasis. There is currently no available treatment for RP. In this review, we discuss the different approaches currently being investigated for treatment of rhodopsin RP, focusing on the potential of manipulation of the proteostasis network as a therapeutic approach to combat retinal degeneration.
视紫红质突变是视网膜色素变性(RP)最常见的病因之一。视紫红质的错误折叠会导致细胞蛋白质稳态(即蛋白平衡)的破坏。目前尚无针对RP的有效治疗方法。在本综述中,我们讨论了目前正在研究的治疗视紫红质RP的不同方法,重点关注操纵蛋白平衡网络作为对抗视网膜变性的治疗方法的潜力。