Department of Pharmacology, Case Western Reserve University, Cleveland, OH, USA.
J Neurochem. 2012 Apr;121(1):146-56. doi: 10.1111/j.1471-4159.2012.07647.x. Epub 2012 Feb 9.
All-trans-retinal and its condensation-products can cause retinal degeneration in a light-dependent manner and contribute to the pathogenesis of human macular diseases such as Stargardt's disease and age-related macular degeneration. Although these toxic retinoid by-products originate from rod and cone photoreceptor cells, the contribution of each cell type to light-induced retinal degeneration is unknown. In this study, the primary objective was to learn whether rods or cones are more susceptible to light-induced, all-trans-retinal-mediated damage. Previously, we reported that mice lacking enzymes that clear all-trans-retinal from the retina, ATP-binding cassette transporter 4 and retinol dehydrogenase 8, manifested light-induced retinal dystrophy. We first examined early-stage age-related macular degeneration patients and found retinal degenerative changes in rod-rich rather than cone-rich regions of the macula. We then evaluated transgenic mice with rod-only and cone-like-only retinas in addition to progenies of such mice inbred with Rdh8(-/-) Abca4(-/-) mice. Of all these strains, Rdh8(-/-) Abca4(-/-) mice with a mixed rod-cone population showed the most severe retinal degeneration under regular cyclic light conditions. Intense light exposure induced acute retinal damage in Rdh8(-/-) Abca4(-/-) and rod-only mice but not cone-like-only mice. These findings suggest that progression of retinal degeneration in Rdh8(-/-) Abca4(-/-) mice is affected by differential vulnerability of rods and cones to light.
全反式视黄醛及其缩合产物可以以光依赖的方式引起视网膜变性,并导致人类黄斑疾病的发病机制,如斯塔加特病和年龄相关性黄斑变性。虽然这些有毒的视黄醛副产物起源于杆状和锥状光感受器细胞,但每种细胞类型对光诱导的视网膜变性的贡献尚不清楚。在这项研究中,主要目的是了解杆状细胞或锥状细胞对光诱导的全反式视黄醛介导的损伤更敏感。此前,我们报道过缺乏从视网膜清除全反式视黄醛的酶的小鼠,即 ATP 结合盒转运蛋白 4 和视黄醇脱氢酶 8,表现出光诱导的视网膜营养不良。我们首先检查了早期年龄相关性黄斑变性患者,发现视网膜退行性变化发生在富含杆状细胞而不是富含锥状细胞的黄斑区域。然后,我们评估了仅具有杆状细胞和类似锥状细胞的转基因小鼠,以及这些小鼠与 Rdh8(-/-)Abca4(-/-)小鼠近交的后代。在所有这些品系中,具有混合杆状细胞-锥状细胞群体的 Rdh8(-/-)Abca4(-/-) 小鼠在常规循环光照条件下表现出最严重的视网膜变性。强烈的光照暴露在 Rdh8(-/-)Abca4(-/-)和仅杆状细胞小鼠中诱导急性视网膜损伤,但在仅类似锥状细胞小鼠中没有。这些发现表明,Rdh8(-/-)Abca4(-/-) 小鼠视网膜变性的进展受到杆状细胞和锥状细胞对光的不同易感性的影响。