Histochemical localisation of mitochondrial enzyme activity in human optic nerve and retina
Histochemical localisation of mitochondrial enzyme activity in human optic nerve and retina
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DOI：
10.1136/bjo.83.2.231
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发表时间：
1999-02-01
影响因子：
4.1
通讯作者：
Turnbull, DM
中科院分区：
文献类型：
--
作者：
Andrews, RM;Griffiths, PG;Turnbull, DM
目的-证明人类视神经和视网膜内线粒体酶的定量分布与眼科疾病发病机制的关系。方法-在多器官捐赠时进行眼球摘除，立即整块切除视神经和视神经乳头周围视网膜并冷冻。线粒体酶细胞色素c氧化酶和琥珀酸脱氢酶的反应性证明在系列cryostat切片使用特定的histochemical assays.Results-In视神经的无髓鞘的前板层和层状区域都丰富的细胞色素c氧化酶和琥珀酸脱氢酶。当它们离开筛板时，纤维的髓鞘形成与酶活性的突然降低有关。在视网膜内，高水平的酶活性被发现定位在视网膜神经节细胞和神经纤维层，外丛状层，内段的光感受器，和the retinal pigment epithelium. Conclusions-线粒体酶活性保存在人类视神经和视网膜检索时的多器官捐赠。眼内酶活性的分布对于理解线粒体疾病中所见的眼部受累模式和视神经受累患者中神经节细胞功能障碍的部位具有意义。
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.