Links between electrophysiological and molecular pathology of amyotrophic lateral sclerosis

Katharina A. Quinlan*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

24 Scopus citations

Abstract

Multiple deficits have been described in amyotrophic lateral sclerosis (ALS), from the first changes in normal functioning of the motoneurons and glia to the eventual loss of spinal and cortical motoneurons. In this review, current results, including changes in size, and electrical properties of motoneurons, glutamate excitotoxicity, calcium buffering, deficits in mitochondrial and cellular transport, impediments to proteostasis which lead to stress of the endoplasmic reticulum (ER), and glial contributions to motoneuronal vulnerability are recapitulated. Results are mainly drawn from the mutant SOD1 mouse model of ALS, and emphasis is placed on early changes that precede the onset of symptoms and the interplay between molecular and electrical processes.

Original languageEnglish (US)
Pages (from-to)913-925
Number of pages13
JournalIntegrative and Comparative Biology
Volume51
Issue number6
DOIs
StatePublished - Dec 2011

ASJC Scopus subject areas

  • Animal Science and Zoology
  • Plant Science

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