Fragmentation of the Golgi apparatus of motor neurons in amyotrophic lateral sclerosis (ALS). Clinical studies in ALS of Guam and experimental studies in deafferented neurons and in beta,beta'-iminodipropionitrile axonopathy.

摘要: Previous morphological immunoenzymatic studies with organelle-specific antibodies have disclosed an apparent fragmentation of the Golgi apparatus in large numbers motor neurons 12 cases sporadic, non-Guamanian amyotrophic lateral sclerosis (ALS) three other types neuron disease and one case a mitochondrial myopathy cytochrome c oxidase deficiency. Motor fragmented were moderately atrophic; these cells, discrete immunostained elements organelle twice as many normal neurons, size each element percentage cytoplasmic area occupied by reduced (Am J Pathol 1992, 140: 731-737). In this report we confirmed spinal cord six sporadic Guamanian ALS. four clinical course was 1 to 2 years. The percentages varied from 38 92. additional ALS duration 5 7 years did not show apparatus. two ALS, all ubiquitin-positive skein-like or granular inclusions believed be pathognomonic for had To examine whether results reactions either neuronal deafferentation lesions proximal axons, conducted experimental studies. first study, examined cats deafferented dorsal geniculate nucleus. second rats axonopathy induced beta,beta'-iminodipropionitrile. experiments, studied techniques morphometry, fragmented. Taken together, strongly suggest that represents important perhaps early change may involved pathogenesis is fairly specific easily recognizable marker used together criteria comparisons between human proposed animal models disorder.