A Bietti Crystalline Dystrophy Mouse Model Shows Increased Sensitivity to Light-Induced Injury
| Autor: | Zhiwei Ma, Xiaodong Jiao, Martin-Paul Agbaga, Robert E. Anderson, Haohua Qian, Qian Li, Lijin Dong, J. Fielding Hejtmancik |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2022 |
| Předmět: |
Organic Chemistry
General Medicine Bietti crystalline dystrophy Cyp4v3 mouse model light-induced injury high-fat diet Catalysis Computer Science Applications Inorganic Chemistry Mice Disease Models Animal Retinal Diseases Mutation Retinal Dystrophies Animals Cytochrome P450 Family 4 Physical and Theoretical Chemistry Atrophy Molecular Biology Spectroscopy |
| Zdroj: | International Journal of Molecular Sciences; Volume 23; Issue 21; Pages: 13108 |
| ISSN: | 1422-0067 |
| DOI: | 10.3390/ijms232113108 |
| Popis: | Bietti crystalline corneo-retinal dystrophy (BCD) is an autosomal recessive inherited retinal dystrophy characterized by multiple shimmering yellow-white deposits in the posterior pole of the retina in association with atrophy of the retinal pigment epithelium (RPE), pigment clumps, and choroidal atrophy and sclerosis. Blindness and severe visual damage are common in late-stage BCD patients. We generated a Cyp4v3 knockout mouse model to investigate the pathogenesis of BCD. This model exhibits decreased RPE numbers and signs of inflammation response in the retina. Rod photoreceptors were vulnerable to light-induced injury, showing increased deposits through fundoscopy, a decrease in thickness and a loss of cells in the ONL, and the degeneration of rod photoreceptors. These results suggest that an inflammatory response might be an integral part of the pathophysiology of BCD, suggesting that it might be reasonable for BCD patients to avoid strong light, and the results provide a useful model for evaluating the effects of therapeutic approaches. |
| Databáze: | OpenAIRE |
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