Evidence for autoimmunity in amyotrophic lateral sclerosis

Stanley H. Appel, R. Glenn Smith, J. Engelhardt, Enrico Stefani

Research output: Contribution to journalArticle

45 Citations (Scopus)

Abstract

Although the etiology and pathogenesis of ALS is unknown, increasing evidence supports a role for autoimmune mechanisms in motoneuron degeneration and death. An animal model, experimental autoimmune gray matter disease, can be induced by the inoculation of spinal cord gray matter. The experimental disease is characterized by weakness secondary to the loss of upper and lower motoneurons, accompanied by inflammatory foci within the spinal cord, and IgG at the neuromuscular junction and within UMN and LMN. In human ALS, IgG is present within the UMN and LMN, and T-lymphocytes and activated microglia have been identified within spinal cord gray matter and motor cortex. ALS IgG can passively transfer physiological changes of the neuromuscular junction to mice resulting in enhanced release of acetylcholine. The ALS IgG selectively interact with calcium channels and alter channel function. These data suggest a potential role for autoimmune mechanisms in the destruction and loss of motoneurons in ALS.

Original languageEnglish
Pages (from-to)14-19
Number of pages6
JournalJournal of the Neurological Sciences
Volume124
Issue numberSUPPL.
DOIs
Publication statusPublished - 1994

Fingerprint

Amyotrophic Lateral Sclerosis
Autoimmunity
Motor Neurons
Immunoglobulin G
Spinal Cord
Neuromuscular Junction
Motor Cortex
Microglia
Calcium Channels
Acetylcholine
Animal Models
T-Lymphocytes
Gray Matter

Keywords

  • ALS
  • Autoimmune
  • Calcium channel
  • Immunoglobulin
  • Motor neuron

ASJC Scopus subject areas

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

Cite this

Evidence for autoimmunity in amyotrophic lateral sclerosis. / Appel, Stanley H.; Smith, R. Glenn; Engelhardt, J.; Stefani, Enrico.

In: Journal of the Neurological Sciences, Vol. 124, No. SUPPL., 1994, p. 14-19.

Research output: Contribution to journalArticle

Appel, Stanley H. ; Smith, R. Glenn ; Engelhardt, J. ; Stefani, Enrico. / Evidence for autoimmunity in amyotrophic lateral sclerosis. In: Journal of the Neurological Sciences. 1994 ; Vol. 124, No. SUPPL. pp. 14-19.
@article{61bbc3647c784e3488690820655c8055,
title = "Evidence for autoimmunity in amyotrophic lateral sclerosis",
abstract = "Although the etiology and pathogenesis of ALS is unknown, increasing evidence supports a role for autoimmune mechanisms in motoneuron degeneration and death. An animal model, experimental autoimmune gray matter disease, can be induced by the inoculation of spinal cord gray matter. The experimental disease is characterized by weakness secondary to the loss of upper and lower motoneurons, accompanied by inflammatory foci within the spinal cord, and IgG at the neuromuscular junction and within UMN and LMN. In human ALS, IgG is present within the UMN and LMN, and T-lymphocytes and activated microglia have been identified within spinal cord gray matter and motor cortex. ALS IgG can passively transfer physiological changes of the neuromuscular junction to mice resulting in enhanced release of acetylcholine. The ALS IgG selectively interact with calcium channels and alter channel function. These data suggest a potential role for autoimmune mechanisms in the destruction and loss of motoneurons in ALS.",
keywords = "ALS, Autoimmune, Calcium channel, Immunoglobulin, Motor neuron",
author = "Appel, {Stanley H.} and Smith, {R. Glenn} and J. Engelhardt and Enrico Stefani",
year = "1994",
doi = "10.1016/0022-510X(94)90171-6",
language = "English",
volume = "124",
pages = "14--19",
journal = "Journal of the Neurological Sciences",
issn = "0022-510X",
publisher = "Elsevier",
number = "SUPPL.",

}

TY - JOUR

T1 - Evidence for autoimmunity in amyotrophic lateral sclerosis

AU - Appel, Stanley H.

AU - Smith, R. Glenn

AU - Engelhardt, J.

AU - Stefani, Enrico

PY - 1994

Y1 - 1994

N2 - Although the etiology and pathogenesis of ALS is unknown, increasing evidence supports a role for autoimmune mechanisms in motoneuron degeneration and death. An animal model, experimental autoimmune gray matter disease, can be induced by the inoculation of spinal cord gray matter. The experimental disease is characterized by weakness secondary to the loss of upper and lower motoneurons, accompanied by inflammatory foci within the spinal cord, and IgG at the neuromuscular junction and within UMN and LMN. In human ALS, IgG is present within the UMN and LMN, and T-lymphocytes and activated microglia have been identified within spinal cord gray matter and motor cortex. ALS IgG can passively transfer physiological changes of the neuromuscular junction to mice resulting in enhanced release of acetylcholine. The ALS IgG selectively interact with calcium channels and alter channel function. These data suggest a potential role for autoimmune mechanisms in the destruction and loss of motoneurons in ALS.

AB - Although the etiology and pathogenesis of ALS is unknown, increasing evidence supports a role for autoimmune mechanisms in motoneuron degeneration and death. An animal model, experimental autoimmune gray matter disease, can be induced by the inoculation of spinal cord gray matter. The experimental disease is characterized by weakness secondary to the loss of upper and lower motoneurons, accompanied by inflammatory foci within the spinal cord, and IgG at the neuromuscular junction and within UMN and LMN. In human ALS, IgG is present within the UMN and LMN, and T-lymphocytes and activated microglia have been identified within spinal cord gray matter and motor cortex. ALS IgG can passively transfer physiological changes of the neuromuscular junction to mice resulting in enhanced release of acetylcholine. The ALS IgG selectively interact with calcium channels and alter channel function. These data suggest a potential role for autoimmune mechanisms in the destruction and loss of motoneurons in ALS.

KW - ALS

KW - Autoimmune

KW - Calcium channel

KW - Immunoglobulin

KW - Motor neuron

UR - http://www.scopus.com/inward/record.url?scp=0027991954&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0027991954&partnerID=8YFLogxK

U2 - 10.1016/0022-510X(94)90171-6

DO - 10.1016/0022-510X(94)90171-6

M3 - Article

VL - 124

SP - 14

EP - 19

JO - Journal of the Neurological Sciences

JF - Journal of the Neurological Sciences

SN - 0022-510X

IS - SUPPL.

ER -