视网膜色素变性(RP)是一种病因和发病机理迄今未明确的眼病,向来被认为是“难治”或“不治”之症。近百年来不少学者做了大量的研究工作,虽然仍没有重大的突破,但是,由于生物化学、免疫学、遗传学以及电生理诊断技术不断进步,使对本病的研究也不断有所进展。这将对进一步认识RP的病因、发病机理,从而对治疗以至优生优育等方面展示了广阔的前景。本文对生化、免疫和遗传三方面加以综述。 Retinitis pigmentosa (RP) is an etiology and pathogenesis has not yet clear eye disease, has always been considered as “refractory” or “dead” disease. In recent centuries, many scholars have done a lot of research work, although there is no major breakthrough, but because of biochemistry, immunology, genetics and electrophysiological diagnostic techniques continue to progress, so that the study of this disease has also continued to progress . This will be further understanding of the etiology and pathogenesis of RP, thus treating and even prenatal and postnatal care and other aspects of the show broad prospects. This article reviews biochemistry, immunity and genetics.