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Histochemical localisation of mitochondrial enzyme activity in human optic nerve and retina

机译:人视神经和视网膜中线粒体酶活性的组织化学定位

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摘要

AIMS—To demonstrate the quantitative distribution of mitochondrial enzymes within the human optic nerve and retina in relation to the pathogenesis of ophthalmic disease.
METHODS—Enucleations were performed at the time of multiple organ donation and the optic nerve and peripapillary retina immediately excised en bloc and frozen. Reactivities of the mitochondrial enzymes cytochrome c oxidase and succinate dehydrogenase were demonstrated in serial cryostat sections using specific histochemical assays.
RESULTS—In the optic nerve the unmyelinated prelaminar and laminar regions were rich in both cytochrome c oxidase and succinate dehydrogenase. Myelination of fibres as they exited the lamina cribrosa was associated with an abrupt reduction in enzyme activity. Within the retina, high levels of enzyme activity were found localised within the retinal ganglion cells and nerve fibre layer, the outer plexiform layer, inner segments of photoreceptors, and the retinal pigment epithelium.
CONCLUSIONS—Mitochondrial enzyme activity is preserved in human optic nerve and retina retrieved at the time of multiple organ donation. The distribution of enzyme activity within the eye has implications for the understanding of the pattern of ophthalmic involvement seen in mitochondrial diseases and the site of ganglion cell dysfunction in those patients with optic nerve involvement.

Keywords: mitochondria; optic nerve; retina
机译:目的—目的是证明与眼病发病机理相关的人类视神经和视网膜中线粒体酶的定量分布。
方法—捐献多器官时进行去核,并立即摘除视神经和乳头周围视网膜整体切除并冷冻。使用特定的组织化学分析方法,在连续低温恒温器切片中证明了线粒体酶细胞色素c氧化酶和琥珀酸脱氢酶的反应性。纤维离开筛板时的髓鞘形成与酶活性的突然降低有关。在视网膜内,发现高水平的酶活性位于视网膜神经节细胞和神经纤维层,外丛状层,光感受器的内部区段以及视网膜色素上皮细胞内。
结论—线粒体酶的活性被保留在捐献多器官时恢复了人类的视神经和视网膜。眼内酶活性的分布对于理解线粒体疾病的眼科受累模式和视神经受累患者的神经节细胞功能障碍部位具有重要意义。

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