Metabolic studies on retinal tissue from a donor with a dominantly inherited chorioretinal degeneration resembling sectoral retinitis pigmentosa.
Metabolic studies on retinal tissue from a donor with a dominantly inherited chorioretinal degeneration resembling sectoral retinitis pigmentosa.
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对患有显性遗传性脉络膜视网膜变性（类似于扇形性视网膜色素变性）的供体的视网膜组织进行代谢研究。
DOI：
10.1016/s0161-6420(84)34311-1
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发表时间：
1984
期刊：
影响因子：
13.7
通讯作者：
R. Ulshafer
中科院分区：
文献类型：
--
作者：
J. Hollyfield;J. Frederick;G. A. Tabor;R. Ulshafer
对一名患有扇形性视网膜色素变性 (RP) 的 79 岁女性的视网膜组织进行体外标记后，使用生化和放射自显影技术监测蛋白质合成、糖基化、RNA 合成和神经递质摄取。对 RP 视网膜的退化和非退化区域以及来自年龄和死后匹配供体的正常视网膜组织进行了比较。 3 H-尿苷孵育后来自RP眼的非退化视网膜的放射自显影显示，与正常对照视网膜相比，所有视网膜层中的细胞核上的银颗粒密度几乎相同。相反，在 3 H-亮氨酸孵育后，注意到非退化RP视网膜中银颗粒密度的光感受器特异性降低。在正常视网膜中，用 3 H-甘露糖标记的视杆光感受器始终大于视锥细胞光感受器标记。这种掺入模式在 RP 视网膜的非简并区域中被逆转，其中杆状光感受器标记不如视锥光感受器观察到的明显。在 RP 视网膜的非简并区域中，观察到 Müller 细胞显着积累 3H-GABA。很少有细胞表现出对 3 H-蝇蕈醇（一种 GABA 类似物）的选择性摄取，表明很少有 GABA 能神经元保留在退化视网膜中。在非退化 RP 视网膜中的常见位置中观察到 3 H-多巴胺积累细胞末端。在RP视网膜的退化区域，注意到3H-GABA和3>H-蝇蕈醇分别被大量和扩散摄取到广泛的细胞过程中，而3>H-多巴胺仅由少数点状末端积累。
Protein synthesis, glycosylation, RNA synthesis, and neurotransmitter uptake were monitored using biochemical and autoradiographic techniques following in vitro labeling of retinal tissue from a 79-year-old female with sectoral retinitis pigmentosa (RP). Comparisons were made between degenerate and non-degenerate regions of the RP retina, and normal retinal tissues from an age- and postmortem-matched donor. Autoradiographs of non-degenerate retina from the RP eye following3>H-uridine incubation revealed virtually identical silver grain density over nuclei in all retinal strata as compared to normal control retinas. In contrast, a photoreceptor-specific reduction in silver grain density in the non-degenerate RP retina was noted following3H-leucine incubation. In the normal retina, rod photoreceptor labeling with3H-mannose was always greater than cone photoreceptor labeling. This pattern of incorporation was reversed in the non-degenerate region of the RP retina where rod photoreceptor labeling was less pronounced than that observed for cone photoreceptors.In non-degenerate regions of the RP retina, a marked accumulation of3H-GABA by the Mller's cells was observed. Few cells exhibited selective uptake of3>H-muscimol, a GABA analog, indicating that few GABAergic neurons remained in the degenerate retina.3H-dopamine-accumulating cell terminals were observed in the usual positions in the non-degenerate RP retina. In the degenerate region of the RP retina, heavy and diffuse uptake of3H-GABA and3>H-muscimol, respectively, into broad cellular processes were noted, whereas3>H-dopamine was accumulated by only a few punctate terminals.