OPA1 expression in the normal rat retina and optic nerve
OPA1 expression in the normal rat retina and optic nerve
复制标题
DOI：
10.1002/cne.20586
复制
发表时间：
2005-07-18
影响因子：
2.5
通讯作者：
Bossy-Wetzel, E
中科院分区：
文献类型：
--
作者：
Ju, WK;Misaka, T;Bossy-Wetzel, E
常染色体显性视神经萎缩（DOA）是遗传性视神经病的最常见形式。 DOA 出现在生命的第一个十年，表现为进行性视力丧失。在 DOA 中，视网膜神经节细胞和视神经因未知机制而退化。 DOA 中的突变基因，即 1 型视神经萎缩 (OPA1)，编码一种与线粒体融合以及线粒体网络和基因组维护有关的动力相关 GTP 酶。在这里，我们确定了正常视网膜和视神经中哪些细胞类型表达OPAL。在正常大鼠视网膜中，OPA1在神经节细胞层以及外丛状层、内核层和内丛状层中表达。在神经节细胞层中，OPA1 主要在视网膜神经节细胞中表达。相比之下，视神经星形胶质细胞和少突胶质细胞中的 OPA1 蛋白含量较低或检测不到。此外，OPA1 蛋白存在于轴突线粒体中。最后，OPA1 表达存在于纯化的视网膜神经节细胞和 RGC-5 细胞系的突起和细胞体的线粒体中。因此，OPA1主要在正常大鼠视网膜的视网膜神经节细胞和视神经的轴突中表达。这些发现可能解释了视网膜神经节细胞对 OPA1 功能丧失的选择性脆弱性。 (c) 2005 年 Wiley-Liss, Inc.
Autosomal dominant optic atrophy (DOA) is the most common form of hereditary optic neuropathy. DOA presents in the first decade of life and manifests as progressive vision loss. In DOA retinal ganglion cells and the optic nerve degenerate by an unknown mechanism. The gene mutated in DOA, Optic Atrophy Type 1 (OPA1), encodes a dynamin-related GTPase implicated in mitochondrial fusion and maintenance of the mitochondrial network and genome. Here, we determine which cell types in the normal retina and the optic nerve express OPAL In the normal rat retina, OPA1 is expressed in the ganglion cell layer as well as in the outer plexiform layer, the inner nuclear layer, and the inner plexiform layer. In the ganglion cell layer, OPA1 is expressed predominantly in retinal ganglion cells. By contrast, OPA1 protein is low or undetectable in astrocytes and oligodendrocytes of the optic nerve. Additionally, OPA1 protein is present in axonal mitochondria. Last, OPA1 expression is present in mitochondria of processes and cell bodies of purified retinal ganglion cells and of the RGC-5 cell line. Thus, OPA1 is predominantly expressed in retinal ganglion cells of the normal rat retina and axons of the optic nerve. These findings may explain the selective vulnerability of retinal ganglion cells to OPA1 loss of function. (c) 2005 Wiley-Liss, Inc.