First published online June 8, 2006
Development 133, 1306e (2006)
© The Company of Biologists Limited
Crystallin clear vision
Cataracts - cloudy lenses - are the leading cause of human blindness. How
cataracts form is poorly understood although defects in crystallins, the major
soluble proteins in lens fibre cells, have been implicated. Now on
p. 2585, Goishi and
colleagues propose that the defective expression of the protein chaperone
A-crystallin causes the structural protein
-crystallin to become
insoluble and blocks the terminal differentiation of lens fibre cells.
Together, these changes cause cataracts. The researchers began their study
when they noticed that zebrafish cloche mutants, which lack blood
cells and blood vessels, also have cataracts. A proteomics analysis indicated
that
-crystallin is insoluble in the cataracts of cloche
mutants, which led the researchers to discover that
A-crystallin is
missing from cloche lenses during their development. The
overexpression of exogenous
A-crystallin rescued the cloche
lens phenotype - one of the first times that cataract formation has been
blocked in vivo. Thus, as well as providing insights into cataract formation,
zebrafish cloche mutants could be used to screen for anti-cataract
drugs.

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Related articles in Development:
A-crystallin expression prevents
-crystallin insolubility and cataract formation in the zebrafish cloche mutant lens
- Katsutoshi Goishi, Akio Shimizu, Gabriel Najarro, Sumiko Watanabe, Rick Rogers, Leonard I. Zon, and Michael Klagsbrun
Development 2006 133: 2585-2593.
[Abstract]
[Full Text]