Purpose.Mature-onset cataract results from the formation of light-scattering aggregates of lens crystallins. Although oxidative or mutational damage may be a prerequisite, little is known of the initiation or nucleation of these aggregated states. In mice carrying mutations in γ-crystallin genes, a truncated form of γ-crystallin formed intranuclear filamentous inclusions within lens fiber cells. Previous studies have shown that bovine crystallins and human γD-crystallin form amyloid fibrils under denaturing conditions in vitro. The amyloid fibril formation of human γC-crystallin (HγC-Crys) induced by low pH, together with characterization of a partially unfolded intermediate in the process were investigated.
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