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Semin Cell Dev Biol. 2000 Feb;11(1):53-60.

The function of alpha-crystallin in vision.

Author information

1
Jules Stein Eye Institute, UCLA School of Medicine, Los Angeles, CA 90095, USA.

Abstract

The alpha-crystallins account for approximately one-third of the total soluble protein in the lens, contributing to its refractive power. 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.

PMID:
10736264
DOI:
10.1006/scdb.1999.0351
[Indexed for MEDLINE]

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