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Review
. 2001 Mar:2 Suppl 1:S47-54.

Calcium: the Darth Vader of ALS

S H Appel  1 , D Beers, L Siklos, J I Engelhardt, D R Mosier
Affiliations
  • PMID: 11465925
Review

Calcium: the Darth Vader of ALS

S H Appel et al. Amyotroph Lateral Scler Other Motor Neuron Disord. 2001 Mar.

Abstract

Motor neuron dysfunction and loss in amyotrophic lateral sclerosis (ALS) have been attributed to several different mechanisms, including increased intracellular calcium, glutamate excitotoxicity, oxidative stress and free radical damage, mitochondrial dysfunction, and neurofilament aggregation and dysfunction of transport mechanisms. These alterations are not mutually exclusive, and increased calcium could be a common denominator. Furthermore, the selective vulnerability of spinal motor neurons and the relative sparing of eye motor neurons represent striking features of both sporadic and familial ALS. Here we review the evidence that calcium homeostasis is altered in ALS, and that low levels of the calcium binding proteins parvalbumin and calbindin-D28K contribute to selective vulnerability by decreasing the ability of motor neurons to handle an increased calcium load, with cell injury and death as the consequence.

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