Mourelatos Z, Yachnis A, Rorke L, Mikol J, Gonatas N K
Department of Pathology, University of Pennsylvania School of Medicine, Philadelphia.
Ann Neurol. 1993 Jun;33(6):608-15. doi: 10.1002/ana.410330609.
The Golgi apparatus plays a key role in the posttranslational processing of polypeptides destined for secretion, incorporation into plasma membranes, and fast axoplasmic transport. Dispersion or fragmentation of the Golgi apparatus, experimentally induced by microtubule-disrupting agents, is associated with decreased secretion of immunoglobulins and insulin. The Golgi apparatus is also involved in targeting of lysosomal enzymes and in the endocytosis of certain hormones, receptors, and toxins. There is a paucity of information on this important organelle in human neuropathological conditions. Using an organelle-specific antiserum we have examined by immunocytochemistry the Golgi apparatus of motor neurons in the spinal cord in 4 patients with amyotrophic lateral sclerosis and 1 patient with Werdnig Hoffmann's disease, 1 with infantile neuronal degeneration, 1 with adult-type familial bulbospinal atrophy, 1 with mitochondrial myopathy with cytochrome c oxidase deficiency, 1 with centronuclear myopathy, and 1 with Duchenne's muscular dystrophy, and in 9 age-matched control subjects. In all motor neuronopathies examined and in the patient with mitochondrial myopathy, 20 to 85% of neurons counted had "fragmented" Golgi apparatus. In age-matched control subjects and the other 2 patients with myopathies, 0 to 1.65% of motor neurons had fragmented Golgi apparatus. These findings suggest that the Golgi apparatus of motor neurons is involved in patients with amyotrophic lateral sclerosis and related motor neuron diseases, and perhaps in patients with certain fatal primary myopathies.
高尔基体在多肽的翻译后加工过程中起着关键作用，这些多肽 destined for secretion（用于分泌）、整合到质膜中以及快速轴浆运输。由微管破坏剂实验诱导的高尔基体分散或碎片化与免疫球蛋白和胰岛素分泌减少有关。高尔基体还参与溶酶体酶的靶向运输以及某些激素、受体和毒素的内吞作用。在人类神经病理状况下，关于这个重要细胞器的信息匮乏。我们使用一种细胞器特异性抗血清，通过免疫细胞化学方法检查了4例肌萎缩侧索硬化症患者、1例韦尔尼克 - 霍夫曼病患者、1例婴儿神经元变性患者、1例成人型家族性延髓脊髓萎缩患者、1例细胞色素c氧化酶缺乏的线粒体肌病患者、1例中央核性肌病患者和1例杜兴氏肌营养不良患者脊髓中运动神经元的高尔基体，以及9例年龄匹配的对照受试者。在所检查的所有运动神经元病患者以及线粒体肌病患者中，计数的神经元中有20%至85%的高尔基体“碎片化”。在年龄匹配的对照受试者和另外2例肌病患者中，0至1.65%的运动神经元高尔基体碎片化。这些发现表明，运动神经元的高尔基体与肌萎缩侧索硬化症及相关运动神经元疾病患者有关，或许也与某些致命的原发性肌病患者有关。 （注：“destined for secretion”原英文表述有误，正确的应该是“destined for secretion”，这里按照正确的英文翻译为“用于分泌” ）
