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

来源：

SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY | 2000年 / 11卷 / 01期

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

页码：53 / 60

页数：8

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