The function of alpha-crystallin in vision.
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.
Horwitz
J
J
Jules Stein Eye Institute, UCLA School of Medicine, Los Angeles, CA 90095, USA.
D000818:Animals; D003459:Crystallins / Q000235:genetics / Q000494:pharmacology / Q000502:physiology*; D006801:Humans; D007908:Lens, Crystalline / Q000737:chemistry; D018832:Molecular Chaperones; D009154:Mutation; D014785:Vision, Ocular / Q000187:drug effects / Q000502:physiology*
D003459:Crystallins; D018832:Molecular Chaperones