The Golgi apparatus of moptor neurons in amyotrophic lateral sclerosis

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Abstract
The Golgi apparatus plays a key role in the posttranslational processing of ploypeptides destined for secretion, incorporation into plasma memberanes, and fast axoplasmic transport. Dispersion of fragmentation of the Golgi apparatus, experimentally induced by microtuble‐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 Werding Hoffmann's disease, I with infantile neuronal degeneration, 1 with adult‐type familial bulbospinal atrophy, I with motochondrial myopathy with cytochrome c oxidase deficiency, 1 with centronuclear myopathy, and 1 with Duchenne'smuscular 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 relation motor neuron diseases, and perhaps in patients with certain fatal primary myopathies.