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Keratocan-deficient mice display alterations in corneal structure
Chia-Yang Liu1, David E Birk, John R Hassell
1Bascom Palmer Eye Institute, Department of Ophthalmology, University of Miami School of Medicine, McKnight Vision Research Center, 1638 NW 10th Avenue, Rm. 621, Miami, FL 33136, USA. cliu2@med.miami.edu
The Journal of Biological Chemistry
|April 1, 2003
Summary
Keratocan (Kera) is vital for maintaining corneal shape and vision. Gene knockout mice show altered collagen structure, impacting corneal shape without affecting transparency.
Area of Science:
- Ophthalmology
- Molecular Biology
- Biochemistry
Background:
- Keratocan (Kera) is a cornea-specific keratan sulfate proteoglycan (KSPG) crucial for corneal transparency.
- Mutations in the KERA gene are linked to cornea plana (CNA2), causing vision loss due to corneal flattening.
Purpose of the Study:
- To investigate the biological role of the Kera gene.
- To establish an animal model for cornea plana.
Main Methods:
- Gene targeting to generate Kera knockout mice (Kera-/-).
- Molecular analyses (Northern, Western blotting, immunohistochemistry) to assess Kera expression.
- Histological and ultrastructural analyses (transmission electron microscopy) to evaluate corneal structure.
Main Results:
- Kera knockout mice lacked Kera mRNA and protein.
- Corneal transparency was maintained, but stromal thickness and cornea-iris angle were reduced.
- Ultrastructural analysis revealed larger stromal fibril diameters and disorganized collagen packing in Kera-/- mice.
Conclusions:
- Keratocan plays a unique role in maintaining corneal shape and collagen matrix organization.
- Ablation of Kera leads to subtle structural changes without compromising corneal transparency in mice.
- Kera is essential for normal corneal curvature and anterior segment geometry.