Unfolding retinal dystrophies: a role for molecular chaperones?

Inherited retinal dystrophy is a major cause of blindness worldwide. Recent molecular studies have suggested that protein folding and molecular chaper ones might play a major role in the pathogenesis of these degenerations. In correct protein folding could be a common consequence of causative mutation s in retinal degeneration disease genes, particularly mutations in the visu al pigment rhodopsin. Furthermore, several retinal degeneration disease gen es have recently been identified as putative facilitators of correct protei n folding, molecular chaperones, on the basis of sequence homology. We also consider whether manipulation of chaperone levels or chaperone function mi ght offer potential novel therapies for retinal degeneration.