Abstract
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.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of the Neurological SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Immunocytochemical visualization of the Golgi apparatus in several species, including human, and tissues with an antiserum against MG-160, a sialoglycoprotein of rat Golgi apparatus.J Histochem Cytochem. 1990; 38: 957-963
- Amyotrophic lateral sclerosis and structural defects in Cu/Zn superoxide dismutase.Science. 1993; 261: 1047-1051
- Progress in unraveling pathways of Golgi traffic.Annu Rev Cell Biol. 1985; 1: 447-488
- MG-160: A novel sialoglycoprotein of the medial cisternae of the Golgi apparatus.J Biol Chem. 1989; 264: 646-653
- Fragmentation of the Golgi apparatus of motor neurons in amyotrophic lateral sclerosis.Am J Pathol. 1992; 140: 731-737
- Contributions to the Physiology of the Golgi apparatus.Am J Pathol. 1994; 145: 751-761
- Motor neuron degeneration in mice that express a human Cu, Zn superoxide mutation.Science. 1994; 264: 1772-1775
- Evidence that all newly synthesized proteins destined for fast axonal transport pass through the Golgi apparatus.J Cell Biol. 1982; 93: 568-575
- Pathogenetic mechanisms in amyotrophic latereal sclerosis and other motor neuron disorders.in: Calne D.B. Neurodegenerative Diseases. Saunders WB, Philadelphia1994: 473-488
- Immunocytochemical and ultrastructural studies of upper motor neurons in amyotrophic lateral sclerosis.Acta Neuropathol. 1992; 83: 518-524
- The Golgi complex: in vitro veritas?.Cell. 1992; 68: 829-840
- Fragmentation of the Golgi apparatus of motor neurons in amyotrophic lateral sclerosis revealed by organelle-specific antibodies.Proc Natl Acad Sci USA. 1990; 87: 4393-4395
- The Golgi apparatus of motor neurons in amyotrophic lateral sclerosis.Ann Neurol. 1993; 33: 608-615
- 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 β,β’ Iminidipropionitrile axonopathy.Am J Path. 1994; 144: 1288-1300
- The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu,Zn superoxide dismutase (SOD) becomes fragmented in early, preclinical stages of the disease.Proc Natl Acad Sci USA. 1996; 93: 5472-5477
- Patterns of neuronal degeneration in motor cortex of amyotrophic lateral sclerosis patients.Acta Neuropathol. 1993; 86: 55-64
- Widely distributed Bunina bodies and spheroids in a case of atypical sporadic amyotrophic lateral sclerosis.Acta Neuropathol. 1991; 81: 349-353
- Bunina bodies in amyotrophic lateral sclerosis immunostained with rabbit anti-cystatin C serum.Neurosci Lett. 1993; 162: 125-128
- Oculomotor nuclear pathology in amyotrophic lateral sclerosis.Acta Neuropathol. 1993; 85: 458-462
- Ubiquitin-positive intraneuronal inclusions in the extramotor cortices of presenile dementia patients with motor neuron disease.J Neurol. 1992; 239: 426-430
- A quantitative electron microscopic study of the intracellular localization of wheat germ agglutinin in retinal neurons.J Comp Neurol. 1986; 254: 287-296
- Transneuronally transported wheat germ agglutinin labels glia as well as neurons in the rat visual system.J Comp Neurol. 1987; 261: 460-465
- Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis.Nature. 1993; 362: 59-62
- Immunocytochemical and ultrastructural studies of the motor cortex in amyotrophic lateral sclerosis.Acta Neuropathol. 1994; 87: 578-585
- The fragmented neuronal Golgi apparatus in amyotrophic lateral sclerosis includes the trans-Golgi-network:functional implications.Acta Neuropathol. 1998; 95: 245-253
Article info
Publication history
Accepted:
January 4,
1999
Received in revised form:
December 23,
1998
Received:
July 10,
1998
Identification
Copyright
© 1999 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
