, Volume 8, Issue 1, pp 279-287
Date: 03 May 2011

Quantity and Activation of Myofiber-Associated Satellite Cells in a Mouse Model of Amyotrophic Lateral Sclerosis

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Introduction

Amyotrophic lateral sclerosis (ALS) is an adult onset motor neuron disease characterized by loss of motor neurons, progressive muscle atrophy, paralysis and ultimately, death. Although great majority of ALS cases are sporadic, 10% are inherited (familial ALS, fALS). Approximately 10%–15% of fALS cases are caused by mutations in Cu/Zn superoxide dismutase gene (SOD1) [1]. SOD1 gene mutations described have permitted the generation of animal models that reproduce the main hallmarks of the human disease [24]. The most commonly used ALS model is an overexpresser of human SOD1 carrying a mutation that substitutes a conserved glycine to alanine (SOD1-G93A) [5]. The SOD1-G93A animals reach symptomatic stage at approximately 90 days of age, showing signs of hindlimb weakness, impaired leg extension and shortened stride length. This proceeds to a complete paralysis of the limbs and to death by day 120–130 of age [6, 7] .

Although the most representative pathological consequence of A ...