總結(jié) |
Rhodopsin is a photoreceptor protein found in retinal rods. It is acomplex formed by the binding of retinaldehyde, the oxidized form of retinol, to the protein opsinand undergoes a series of complex reactions in response to visible light resulting in thetransmission of nerve impulses to the brain. Mutation of the rhodopsin gene is a majorcontributor to various retinopathies such as retinitis pigmentosa. The disease-causing proteingenerally aggregates with ubiquitin in inclusion bodies, disrupts the intermediate filamentnetwork and impairs the ability of the cell to degrade non-functioning proteins which leads tophotoreceptor apoptosis (Berson et al., 1991). Other mutations on rhodopsin lead to X-linkedcongenital stationary night blindness, mainly due toconstitutive activation, when the mutationsoccur around the chromophore binding pocket of rhodopsin (Dryja et al.,1993). Several otherpathological states relating to rhodopsin have been discovered including poor post-Golgitrafficking, dysregulative activation, rod outer segment instability and arrestin binding.
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