The function of alpha-crystallin in vision

被引:167
作者
Horwitz, J [1 ]
机构
[1] Univ Calif Los Angeles, Sch Med, Jules Stein Eye Inst, Los Angeles, CA 90095 USA
关键词
alpha-crystallin; small heat shock protein; cataract; aggregation; molecular chaperones;
D O I
10.1006/scdb.1999.0351
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
The alpha-crystallins account for approximately one-third of the total soluble protein in the lens, contributing to its refractive pourer. In addition, alpha-crystallin also has a chaperone-like function and thus can bind unfolding lens proteins. Alpha B-crystallin is also found outside the lens, having an extensive tissue distribution. It is over-expressed in response to stresses of all kinds, where it is thought to serve a general protective function. Recently, it has been shown in humans that naturally occurring point mutations in the alpha-crystallins result in a deficit in chaperone-like function, and cause cataracts as well as a desmin-related myopathy. This review summarizes much of the past and current knowledge concerning the structure and functions of alpha-crystallin.
引用
收藏
页码:53 / 60
页数:8
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