Fragmentation of the Golgi apparatus of the anterior horn cells in patients with familial amyotrophic lateral sclerosis with SOD1 mutations and posterior column involvement

Yukio Fujita, Koichi Okamoto, Atsushi Sakurai, Nicholas K. Gonatas, Asao Hirano

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Abstract

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. (C) 2000 Elsevier Science B.V.

Original languageEnglish (US)
Pages (from-to)137-140
Number of pages4
JournalJournal of the Neurological Sciences
Volume174
Issue number2
DOIs
StatePublished - Mar 15 2000

Fingerprint

Anterior Horn Cells
Golgi Apparatus
Mutation
Motor Neurons
Spinal Cord
Sialoglycoproteins
Motor Cortex
Horns
Paralysis
Transgenic Mice
Amyotrophic lateral sclerosis 1
Membranes
Antibodies
Genes

Keywords

  • Familial amyotrophic lateral sclerosis
  • Golgi apparatus
  • MG-160
  • Pathology
  • SOD1 gene
  • Spinal cord

ASJC Scopus subject areas

  • Aging
  • Clinical Neurology
  • Surgery
  • Developmental Neuroscience
  • Neurology
  • Neuroscience(all)

Cite this

Fragmentation of the Golgi apparatus of the anterior horn cells in patients with familial amyotrophic lateral sclerosis with SOD1 mutations and posterior column involvement. / Fujita, Yukio; Okamoto, Koichi; Sakurai, Atsushi; Gonatas, Nicholas K.; Hirano, Asao.

In: Journal of the Neurological Sciences, Vol. 174, No. 2, 15.03.2000, p. 137-140.

Research output: Contribution to journalArticle

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