RETINAL DEGENERATION: A NEW ANIMAL MODEL

人类遗传性视网膜变性知之甚少 起病于早、中、晚年，且经常 向完全失明的方向发展。像视网膜炎和年龄这样的症状- 相关性黄斑病变是该班最常见的疾病。 疾病的威胁。 有人提议用实验来阐明失明的原因。 人类疾病的动物模型。此型号、RD或 鸡的视网膜退行性变，表现出隐性的模式 导致早期失明的缺陷的基因传递 生活。病变始于后眼底的一个斑点，扩散 在外周，有伴随的色素变化和视觉 电生理测试显示细胞有缺陷后，细胞就会死亡。 因此，该模型可作为研究人类的替代模型。 综合症。 初步研究表明，该缺陷导致RD失明 鸡是在视觉传导的层面上：转换 光能转化为电脉冲。目前建议进行的研究 GRANT的目的是检查与可视化有关的几个过程 转导。氧化代谢与视觉色素漂白 将由硝基蓝四氮唑组织化学检查 反应和早期受体电位分别为。在场 或缺乏特定的视网膜蛋白(如视觉色素 蛋白质和G蛋白，将通过免疫细胞化学进行检测 和蛋白质分离技术。我们将确定视紫红质 对光的反应是磷酸化的，比较环状 突变体中的核苷酸水平与正常视力的人相比 小鸡。视蛋白合成和外节更新将是 经过生化和解剖学检查。最后，Na-K- ATPase分子，它控制着一个重要的膜离子 光感受器中的泵浦将被分析为一种可能的 失明的原因/结果。 这些实验将使我们深入了解失明的原因。 并进入视觉传导的正常过程。

Human hereditary retinal degenerations are poorly understood diseases that begin in early, middle or late life and frequently procede to total blindness. Syndromes such as retinitis and age- related maculopathy are the most common disorders in the class of diseases. Experiments are proposed to elucidate the cause of blindness in an animal model for the human diseases. This model, the rd, or retinal degenerate strain of chickens, exhibits a recessive mode of genetic transmission of a defect that leads to blindness early in life. The lesion begins as a locus in the posterior fundus, spreads peripherally, has accompanying pigmentary changes and the visual cells die after electrophysiological tests show them defective. Therefore, this model is useful as a surrogate for studying human syndromes. Preliminary studies suggest that the defect causing blindness in rd chicks is at the level of visual transduction: the conversion of light energy to electrical impulse. Studies proposed in the current grant are aimed at examining several processes involved in visual transduction. Oxidative metabolism and visual pigment bleaching will be examined by the nitroblue tetrazolium histochemical reaction and the early receptor potential, respectively. Presence or absence of specific retinal proteins (such as visual pigment proteins and G-protein, will be examined by immunocytochemistry and protein separation techniques. We will determine if rhodopsin is phosphorylated in response to light and compare cylic nucleotide levels in the mutants to those in normally sighted chicks. Opsin synthesis and outersegment renewal will be examined biochemically and anatomically. Finally, the Na-K- ATPase molecule, which controls an important membrane ion pump in photoreceptors will be analyzed, as a possible cause/effect of the blindness. These experiments will provide insight into the cause of blindness and into the normal processes of visual transduction.