| First Author | Mittag TW | Year | 1999 |
| Journal | Exp Eye Res | Volume | 69 |
| Issue | 6 | Pages | 677-83 |
| PubMed ID | 10620397 | Mgi Jnum | J:59356 |
| Mgi Id | MGI:1351423 | Doi | 10.1006/exer.1999.0748 |
| Citation | Mittag TW, et al. (1999) Light-induced retinal damage in mice carrying a mutated SOD I gene. Exp Eye Res 69(6):677-83 |
| abstractText | Transgenic mice expressing mutated mouse Cu/Zn superoxide dismutase (SOD I), corresponding to a mutation associated with familial amyotrophic lateral sclerosis, develop a fatal motorneuron degeneration that resembles the human disease. The biochemical properties of some mutant SOD I enzymes indicate that a gain of catalytic functions, (such as increased peroxidase activity) may be the pathologic factor(s). However, at the present time there is little in vivo evidence that a mutation-induced change in the catalytic activity of SOD I is directly involved in neuronal cell death or that vulnerability to cell death is related to the level of functional/metabolic activity of cells carrying mutated SOD I. In pigmented mice carrying the G86R mutation of mouse SOD I, exposure to constant bright light for 20 days caused a diminution of electroretinographic activity and specific degeneration of photoreceptor cells, while no pathological effects were seen in transgenic littermates not exposed to bright light or in light exposed non-transgenic littermates. These findings are the first to indicate that one mechanism for neuronal cell death by mutated SOD I is use-dependent and/or related to metabolic activity, and therefore may be due to a gain in function of catalytic activities involving superoxide/hydrogen peroxide. The light-exposure pathology in this transgenic mouse model indicates an essential role for SOD I in the protection of photoreceptors from light-damage. Copyright 1999 Academic Press. |
