Article
Expression of Cataract-linked γ-Crystallin Variants in Zebrafish Reveals a Proteostasis Network That Senses Protein Stability.
The Journal of biological chemistry - 2 Dec 2016
Wu Shu-Yu, Zou Ping, Fuller Alexandra W, Mishra Sanjay, Wang Zhen, Schey Kevin L, Mchaourab Hassane S
Abstract excerpt
The refractivity and transparency of the ocular lens is dependent on the stability and solubility of the crystallins in the fiber cells. A number of mutations of lens crystallins have been associated with dominant cataracts in humans and mice. Of particular interest were γB- and γD-crystallin mutants linked to dominant cataracts in mouse models. Although thermodynamically destabilized and aggregation-prone, these...
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