Motoneuron disease: Basic science

Hristelina Ilieva, Nicholas J Maragakis

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

ALS is a relentless neurodegenerative disease in which motor neurons are the susceptible neuronal population. Their death results in progressive paresis of voluntary and respiratory muscles. The unprecedented rate of discoveries over the last two decades have broadened our knowledge of genetic causes and helped delineate molecular pathways. Here we critically review ALS epidemiology, genetics, pathogenic mechanisms, available animal models, and iPS cell technologies with a focus on their translational therapeutic potential. Despite limited clinical success in treatments to date, the new discoveries detailed here offer new models for uncovering disease mechanisms as well as novel strategies for intervention.

Original languageEnglish (US)
Title of host publicationAdvances in Neurobiology
PublisherSpringer New York LLC
Pages163-190
Number of pages28
Volume15
DOIs
StatePublished - 2017

Publication series

NameAdvances in Neurobiology
Volume15
ISSN (Print)2190-5215

Keywords

  • Animal models
  • C9ORF72
  • Epidemiology
  • FUS
  • Genetics
  • iPSC
  • Molecular mechanisms
  • SOD1
  • TDP-43

ASJC Scopus subject areas

  • Biochemistry
  • Neurology
  • Developmental Neuroscience
  • Cellular and Molecular Neuroscience

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  • Cite this

    Ilieva, H., & Maragakis, N. J. (2017). Motoneuron disease: Basic science. In Advances in Neurobiology (Vol. 15, pp. 163-190). (Advances in Neurobiology; Vol. 15). Springer New York LLC. https://doi.org/10.1007/978-3-319-57193-5_6