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Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disorder of motor neurones. Although the genetic basis of familial forms of ALS has been well explored, the molecular basis of sporadic ALS is less well understood. Recent evidence has linked sporadic ALS with the failure to edit key residues in ionotropic glutamate receptors, resulting in excessive influx of calcium ions into motor neurones which in turn triggers cell death. Here we suggest that edited AMPA glutamate (GluR2) receptor subunits serve as gatekeepers for motor neurone survival.

Original publication

DOI

10.1002/bies.20836

Type

Journal article

Journal

Bioessays

Publication Date

11/2008

Volume

30

Pages

1185 - 1192

Keywords

Amyotrophic Lateral Sclerosis, Animals, Calcium, Humans, Ions, Models, Biological, Motor Neurons, Neurons, Permeability, Protein Biosynthesis, RNA Editing, Receptors, AMPA, Transcription, Genetic