TY - JOUR
T1 - Experimental immune-mediated motor neuron diseases
T2 - Models for human ALS
AU - Smith, R. Glenn
AU - Engelhardt, Josef I.
AU - Tajti, Janos
AU - Appel, Stanley H.
N1 - Copyright:
Copyright 2014 Elsevier B.V., All rights reserved.
PY - 1993
Y1 - 1993
N2 - 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.
AB - 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.
KW - ALS
KW - Autoimmune
KW - Calcium channel
KW - Immunoglobulin
KW - Motoneurone
KW - Passive transfer
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U2 - 10.1016/0361-9230(93)90268-G
DO - 10.1016/0361-9230(93)90268-G
M3 - Article
C2 - 8457886
AN - SCOPUS:0027471318
VL - 30
SP - 373
EP - 380
JO - Brain Research Bulletin
JF - Brain Research Bulletin
SN - 0361-9230
IS - 3-4
ER -