Temporospatial analysis and new players in the immunology of amyotrophic lateral sclerosis

Abhirami K. Iyer, Kathryn Jones, Virginia M. Sanders, Chandler Walker

Research output: Contribution to journalReview article

2 Citations (Scopus)

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive loss of lower and upper motor neurons (MN) leading to muscle weakness, paralysis and eventually death. Although a highly varied etiology results in ALS, it broadly manifests itself as sporadic and familial forms that have evident similarities in clinical symptoms and disease progression. There is a tremendous amount of knowledge on molecular mechanisms leading to loss of MNs and neuromuscular junctions (NMJ) as major determinants of disease onset, severity and progression in ALS. Specifically, two main opposing hypotheses, the dying forward and dying back phenomena, exist to account for NMJ denervation. The former hypothesis proposes that the earliest degeneration occurs at the central MNs and proceeds to the NMJ, whereas in the latter, the peripheral NMJ is the site of precipitating degeneration progressing backwards to the MN cell body. A large body of literature strongly indicates a role for the immune system in disease onset and progression via regulatory involvement at the level of both the central and peripheral nervous systems (CNS and PNS). In this review, we discuss the earliest reported immune responses with an emphasis on newly identified immune players in mutant superoxide dismutase 1 (mSOD1) transgenic mice, the gold standard mouse model for ALS.

Original languageEnglish (US)
Article number631
JournalInternational Journal of Molecular Sciences
Volume19
Issue number2
DOIs
StatePublished - Feb 23 2018

Fingerprint

immunology
Immunology
Neuromuscular Junction
Amyotrophic Lateral Sclerosis
Allergy and Immunology
progressions
Neurons
degeneration
Motor Neurons
neurons
mice
Disease Progression
peripheral nervous system
paralysis
Immune system
Neurology
etiology
immune systems
central nervous system
Superoxide Dismutase

Keywords

  • Amyotrophic lateral sclerosis (ALS)
  • Immune response
  • Motor neuron disease
  • Neuroimmunology

ASJC Scopus subject areas

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry

Cite this

Temporospatial analysis and new players in the immunology of amyotrophic lateral sclerosis. / Iyer, Abhirami K.; Jones, Kathryn; Sanders, Virginia M.; Walker, Chandler.

In: International Journal of Molecular Sciences, Vol. 19, No. 2, 631, 23.02.2018.

Research output: Contribution to journalReview article

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