Biochemistry of retinal dystrophy.

The study of progressive hereditary degeneration of the retina in laboratory animals in conditions resembling human retinitis pigmentosa was one of Professor Arnold Sorsby's major research interests during the past three decades. It was in this field of work that I was privileged to be his collaborator for a number of years. The search for a biochemical lesion as the causative agent in such conditions was carried out in the M. R. C. Wernher Research Unit for Ophthalmological Genetics, in London, and encompassed detailed histopathological and biochemical investigations. In addition, the experimental production of similar lesions using specific retinotoxic substances was pursued with great vigour in the hope that knowledge of the pharmacological mode of action of such compounds would offer some means of counteracting their toxic effects. The ultimate aim of such an approach was the provision of therapeutic measures to combat or offset the retinal degenerative process in human retinitis pigmentosa (Sorsby, 1941; Noell, 1953; Edge et al., 1956; Sorsby and Nakajima, 1958a, b; Sorsby and Harding, 1962a, b; Sorsby and Reading, 1964; Reading and Sorsby, 1966b; Reading, 1970). It would be impracticable to review all the work done in all laboratories on this problem, so it is intended in the present paper to describe and review the biochemical and histochemical studies on retinal dystrophies in animals other than man. In addition, an attempt is made to correlate present knowledge to give a rational analysis of the sequential nature of the biochemical lesion which undoubtedly underlies the progress of these genetically determined retinal degenerations.