Experimental immune-mediated motor neuron diseases: Models for human ALS

R. Glenn Smith, J. Engelhardt, J. Tajti, Stanley H. Appel

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

Amyotrophic lateral sclerosis is an idiopathic, utlimately fatal disease, clinically manifest as progressive weakness and spasticity, associated with the loss of motoneurons. Circumstantial evidence supports a role for autoimmune processes in the progression of this human disorder. Two immune-mediated animal models have been developed in our laboratory for motor neuron loss. Experimental autoimmune motor neuron disease is a lower motor syndrome induced in guinea pigs by the repeated injection of a purified bovine spinal motor neuron antigen. Affected animals demonstrate extremity weakness, associated with electromyographic and morphologic evidence of denervation, a loss of spinal cord motor neurons, high antibody titers against motor neurons, and localization of IgG immunoreactivity to the neuromuscular junction and motor neuron cytoplasm. Experimental autoimmune grey matter disease is a more acute and severe disorder involving both upper and lower motor neurons, induced in guinea pigs by inoculation of a bovine ventral spinal cord homogenate, in which scattered foci of denervation are observed in the motor cortex and ventral spinal cord. Similarities between these diseases and human ALS are reviewed.

Original languageEnglish
Pages (from-to)373-380
Number of pages8
JournalBrain Research Bulletin
Volume30
Issue number3-4
DOIs
Publication statusPublished - 1993

Fingerprint

Motor Neuron Disease
Motor Neurons
Spinal Cord
Denervation
Guinea Pigs
Neuromuscular Junction
Amyotrophic Lateral Sclerosis
Motor Cortex
Cytoplasm
Extremities
Animal Models
Immunoglobulin G
Antigens
Injections
Antibodies

Keywords

  • ALS
  • Autoimmune
  • Calcium channel
  • Immunoglobulin
  • Motoneurone
  • Passive transfer

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Experimental immune-mediated motor neuron diseases : Models for human ALS. / Smith, R. Glenn; Engelhardt, J.; Tajti, J.; Appel, Stanley H.

In: Brain Research Bulletin, Vol. 30, No. 3-4, 1993, p. 373-380.

Research output: Contribution to journalArticle

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