Night blindness revisited: from man to molecules. Proctor lecture

Abstract

Night blindness is a frequent concomitant of retinal disorders, many of which are of genetic origin. Through the use of quantitative noninvasive test procedures it has been possible to study patients with these hereditary conditions and to show that the visual abnormalities often result from defects in the functional properties of the rod photoreceptors. More important, the uniqueness of the functional disturbance in the various types of night-blinding disorders suggests that each involves a specific aspect of the rod's internal machinery, i.e., the molecular processes devoted to transduction, intercellular communication, and the renewal of cellular components. Knowledge gained from the study of these clinical entities and from the investigation of experimental animals regarding the cellular events involved in these vital processes have enabled us to formulate tentative hypothesis as to the molecular bases of the hereditary defects.