Early functional deficit and microglial disturbances in a mouse model of amyotrophic lateral sclerosis.

PloS One
Yannick N GerberFlorence E Perrin

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by selective motoneurons degeneration. There is today no clear-cut pathogenesis sequence nor any treatment. However growing evidences are in favor of the involvement, besides neurons, of several partners such as glia and muscles. To better characterize the time course of pathological events in an animal model that recapitulates human ALS symptoms, we investigated functional and cellular characteristics of hSOD1(G93A) mice. We have evaluated locomotor function of hSOD1(G93A) mice through dynamic walking patterns and spontaneous motor activity analysis. We detected early functional deficits that redefine symptoms onset at 60 days of age, i.e. 20 days earlier than previously described. Moreover, sequential combination of these approaches allows monitoring of motor activity up to disease end stage. To tentatively correlate early functional deficit with cellular alterations we have used flow cytometry and immunohistochemistry approaches to characterize neuromuscular junctions, astrocytes and microglia. We show that (1) decrease in neuromuscular junction's number correlates with motor impairment, (2) astrocytes number is not altered at pre- and early-sy...Continue Reading

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Citations

Aug 27, 2014·BioMed Research International·Ana C CalvoRosario Osta
Aug 1, 2015·Molecular Neurodegeneration·Harun Najib NoristaniFlorence Evelyne Perrin
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Methods Mentioned

BETA
transgenic
biopsy
flow cytometry
FACS
transgenics
PCR

Software Mentioned

GraphPad
Adobe® Photoshop®
Bioseb
FACSDiva
Actitrack
GraphPad Prism

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