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Macular corneal dystrophy: immunochemical characterization using monoclonal antibodies
N SundarRaj1, E Barbacci-Tobin, W E Howe
1Department of Ophthalmology, University of Pittsburgh School of Medicine, Pennsylvania 15213.
Investigative Ophthalmology & Visual Science
|October 1, 1987
Summary
Researchers identified a novel antigen in macular corneal dystrophy, distinct from vimentin, which is overexpressed in affected corneas. This finding advances understanding of corneal stromal deposits in inherited eye diseases.
Area of Science:
- Ophthalmology
- Cell Biology
- Biochemistry
Background:
- Macular corneal dystrophy (MCD) is an inherited eye condition causing corneal opacities due to abnormal stromal deposits.
- Understanding the molecular basis of these deposits is crucial for diagnosing and potentially treating MCD.
Purpose of the Study:
- To identify and characterize the specific antigens present in the abnormal stromal deposits of macular corneal dystrophy.
- To differentiate these antigens from known cytoskeletal proteins like vimentin.
Main Methods:
- Screening of monoclonal antibodies against corneal fibroblast antigens.
- Indirect peroxidase-conjugated immunostaining on corneal tissues.
- Immunofluorescent staining and immunoelectron microscopy on cultured corneal fibroblasts.
- Immunoblotting analysis of cytoskeletal proteins.
Main Results:
- A monoclonal antibody (8F1-3) strongly reacted with abnormal deposits in MCD corneas.
- The antigen recognized by 8F1-3 showed increased concentration in MCD corneal cells compared to normal cells.
- Immunostaining revealed a filamentous pattern similar to vimentin, but the antigen was not vimentin.
- Immunoblotting identified two distinct polypeptides (Mr 48,000 and 45,000) recognized by 8F1-3, differing from vimentin (Mr 58,000).
Conclusions:
- A novel intermediate filament-associated antigen, distinct from vimentin, is significantly elevated in macular corneal dystrophy.
- This antigen represents a potential diagnostic marker and therapeutic target for MCD.