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[Ultrastructure and crystallin mutant molecular modeling of hereditary coralliform cataract]
Wei-zhen Xu1, Shu Zheng, Qi Dong
1The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310009, China.
Objective:
To observe the correlation of gammaD-crystallin P23T mutant with lens ultrastructure of the hereditary coralliform cataract.
Methods:
Complete ophthalmologic examinations were performed before lens extraction and lens samples were studied by transmission and scanning electric microscope respectively. Protein molecular modeling was performed using SWISS-MODEL(version 2.0).
Results:
Protein structure modeling demonstrated that the mutant caused a decrease in molecular final total energy and changes in the surface structure of gammaD-crystallin. Ultrastructure study revealed crystals deposited in lens, extensive granules dispersed in uncommon oval structure and the disorganization of lens epithelial cells.
Conclusion:
It is possible that the gammaD-crystallin P23T mutant is associated with abnormal crystals in lens and disorganization of lens epithelial cells.