Fragmentation of the Golgi apparatus of the anterior horn cells in patients with familial amyotrophic lateral sclerosis with SOD1 mutations and posterior column involvement.
The Golgi apparatus (GA) of the anterior horn cells in the spinal cord was examined by immunohistological methods with an antibody against the MG-160 protein, a conserved intrinsic membrane sialoglycoprotein of the medial cisternae of the GA, in three patients with familial amyotrophic lateral sclerosis (FALS) with posterior column involvement. Large motor neurons in the anterior horns were markedly reduced in number and 10 of total 14 remaining large motor neurons showed fragmentation and a reduction in the number of the elements of the GA. The fragmentation of the GA was identical to that previously reported in motor neurons of the spinal cord and motor cortex from patients with sporadic ALS and in transgenic mice expressing the G93A mutation of the gene encoding the Cu/Zn superoxide dismutase months before the onset of paralysis. This is the first report of fragmented GA of the anterior horn cells in patients with FALS with posterior column involvement. The findings suggest that the GA is a common target in the neuronal degeneration in sporadic and FALS.
Fujita
Y
Y
Department of Neurology, Gunma University School of Medicine, 3-39-22, Showa-machi, Maebashi, Gunma, Japan.
Okamoto
K
K
Sakurai
A
A
Gonatas
N K
NK
Hirano
A
A
D019943:Amino Acid Substitution; D000870:Anterior Horn Cells / Q000473:pathology*; D015415:Biomarkers; D003062:Codon / Q000235:genetics; D006056:Golgi Apparatus / Q000648:ultrastructure*; D006801:Humans; D016472:Motor Neuron Disease / Q000201:enzymology / Q000235:genetics / Q000473:pathology*; D009410:Nerve Degeneration; D009419:Nerve Tissue Proteins / Q000032:analysis*; D017354:Point Mutation; D011956:Receptors, Cell Surface; D017468:Receptors, Fibroblast Growth Factor; D012795:Sialoglycoproteins / Q000032:analysis*; D013116:Spinal Cord / Q000473:pathology*; D013482:Superoxide Dismutase / Q000235:genetics*
D015415:Biomarkers; D003062:Codon; D009419:Nerve Tissue Proteins; D011956:Receptors, Cell Surface; D017468:Receptors, Fibroblast Growth Factor; D012795:Sialoglycoproteins; C086711:cysteine-rich fibroblast growth factor receptor; D013482:Superoxide Dismutase