Inherited retinal dystrophies: Cell biology and interacting co-factors

M. Boulton

Research output: Contribution to journalArticle

Abstract

This study was initiated to investigate the relationship between cellular aging and inherited retinal dystrophies. Cultured human retinal pigment epithelial (RPE) cells from 14 dystrophic donors of various genotypes did not exhibit abnormal behavioral characteristics when compared with normal human RPE cell cultures of equivalent age in test conditions. In contrast, behavioral cell characteristics did vary with donor age. Techniques have been developed which permit topographical analysis of RPE cells at different retinal locations. They demonstrate variation in lysosomal enzyme distribution across the retina and provide a model for lipofuscin formation, repigmentation of cultured RPE, and quantification of peroxisomal enzymes. It is suggested that in late onset retinal dystrophies gene defects are contained by repair mechanisms until aging and/or optical radiation damage render these mechanisms incapable of fully compensating for the cumulative effects of all factors.

Original languageEnglish (US)
Pages (from-to)29-39
Number of pages11
JournalChibret International Journal of Ophthalmology
Volume6
Issue number2
StatePublished - Dec 1 1989

ASJC Scopus subject areas

  • Ophthalmology

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