Fragmentation of the Golgi apparatus of Betz cells in patients with amyotrophic lateral sclerosis

• Published in: Journal of the neurological sciences Vol. 163; no. 1; pp. 81 - 85
• Main Authors: Fujita, Yukio, Okamoto, Koichi, Sakurai, Atsushi, Amari, Masakuni, Nakazato, Yoichi, Gonatas, Nicholas K.
• Format: Journal Article
• Language: English
• Published: Shannon Elsevier B.V 01-02-1999; Elsevier Science
• Subjects: Adult; Aged; Aged, 80 and over; Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - pathology; Animals; Betz cell; Betz cells; Biological and medical sciences; Cerebral Cortex - pathology; Cerebral Cortex - ultrastructure; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Female; Golgi apparatus; Golgi Apparatus - pathology; Golgi Apparatus - ultrastructure; Humans; Male; Medical sciences; MG-160 protein; Mice; Mice, Transgenic; Middle Aged; Motor Neurons - pathology; Motor Neurons - ultrastructure; Neurology; Point Mutation; Pyramidal Cells - pathology; Pyramidal Cells - ultrastructure; Superoxide Dismutase - genetics; Amyotrophic lateral sclerosis; MG-160 protein; Betz cell; Golgi apparatus; Human; Immunohistochemistry; Nervous system diseases; Exploration; Betz neuron; Pathology; Ultrastructure; Central nervous system disease; Adult; Degenerative disease; Spinal cord disease; Brain (vertebrata)
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/S0022-510X(99)00014-3

The Golgi apparatus (GA) of the large pyramidal motor neurons in the cerebral cortex (Betz cells), was examined in sixteen patients with sporadic amyotrophic lateral sclerosis (ALS), in one patient with familial ALS (FALS), and in ten non-ALS age matched controls including one patient with Huntington’s disease and one patient with a brain infarct. The GA was immunostained with an antibody against the MG-160 protein, a conserved sialoglycoprotein of the medial cisternae of the organelle. In ALS, 13.2% of counted Betz cells had fragmented GA in contrast to 0.6% in the ten non-ALS controls. The fragmentation of the GA of Betz cells was identical to that previously reported in spinal cord motor neurons from patients with sporadic ALS and in transgenic mice expressing the G93A mutation of the gene encoding the Cu/Zn superoxide dismutase. The striking morphological similarity between the fragmentation of the GA observed in Betz cells and in spinal cord motor neurons suggests that a similar pathogenic mechanism is responsible for both, and that the fragmentation of the GA of the spinal cord motor neurons is not a consequence of deafferentation due to the degeneration of the Betz cells.