Apoptosis, retinitis pigmentosa, and degeneration.
The mechanism of photoreceptor cell death in different inherited retinal degenerations is not fully understood. Mutations in a number of different genes (such as rhodopsin, the beta subunit of cGMP phosphodiesterase, and peripherin) have been identified as the primary genetic lesion in different forms of human retinitis pigmentosa, one of the most common causes of inherited blindness. In all cases the manifestation of the disorder regardless of the specific primary genetic lesion is similar, resulting in photoreceptor cell degeneration and blindness. A recent hypothesis is that the active photoreceptor cell death, which is characteristic of these genetically distinct disorders, is mediated by a common induction of apoptosis. In the present review, the current evidence for active cell death during retinal cell death in several different rodent models of retinitis pigmentosa and retinal degeneration is examined.
Wong
P
P
National Eye Institute, National Institutes of Health, Bethesda, MD 20892, USA.
D000818:Animals; D017209:Apoptosis; D051152:Clusterin; D004195:Disease Models, Animal; D006023:Glycoproteins / Q000502:physiology; D006801:Humans; D018832:Molecular Chaperones; D012160:Retina / Q000254:growth & development / Q000473:pathology*; D012162:Retinal Degeneration / Q000473:pathology*; D012174:Retinitis Pigmentosa / Q000473:pathology*
C495242:CLU protein, human; D051152:Clusterin; D006023:Glycoproteins; D018832:Molecular Chaperones