Other
14 Authors
- Dunaief JL,
- Lee MP,
- Otsu W,
- Fu C,
- Badea TC,
- Hsu KS,
- Nuruzzaman A,
- Akbar AF,
- Chuang JZ,
- Guo Z,
- Nakajima N,
- Yang HH,
- Yang N,
- Sung CH
First Author | Chuang JZ | Year | 2022 |
Journal | Nat Commun | Volume | 13 |
Issue | 1 | Pages | 374 |
PubMed ID | 35042858 | Mgi Jnum | J:357303 |
Mgi Id | MGI:6865007 | Doi | 10.1038/s41467-021-27935-9 |
Citation | Chuang JZ, et al. (2022) Retinal pigment epithelium-specific CLIC4 mutant is a mouse model of dry age-related macular degeneration. Nat Commun 13(1):374 |
abstractText | Age-related macular degeneration (AMD) is the leading cause of blindness among the elderly. Dry AMD has unclear etiology and no treatment. Lipid-rich drusen are the hallmark of dry AMD. An AMD mouse model and insights into drusenogenesis are keys to better understanding of this disease. Chloride intracellular channel 4 (CLIC4) is a pleomorphic protein regulating diverse biological functions. Here we show that retinal pigment epithelium (RPE)-specific Clic4 knockout mice exhibit a full spectrum of functional and pathological hallmarks of dry AMD. Multidisciplinary longitudinal studies of disease progression in these mice support a mechanistic model that links RPE cell-autonomous aberrant lipid metabolism and transport to drusen formation. |