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Pre-symptomatic electrophysiological tests predict clinical disease onset and survival in SOD1G93A ALS mice

Pre-symptomatic electrophysiological tests predict clinical disease onset and survival in SOD1G93A ALS mice
Pre-symptomatic electrophysiological tests predict clinical disease onset and survival in SOD1G93A ALS mice
Introduction: we assessed the predictive value of electrophysiological tests as a marker of clinical disease onset and survival in superoxide-dismutase 1 (SOD1)(G93A) mice.

Methods: we evaluated the accuracy of electrophysiological tests in differentiating transgenic versus wild-type mice. We made a correlation analysis of electrophysiological parameters and the onset of symptoms, survival, and number of spinal motoneurons.

Results: presymptomatic electrophysiological tests show great accuracy in differentiating transgenic versus wild-type mice, with the most sensitive parameter being the tibialis anterior compound muscle action potential (CMAP) amplitude. The CMAP amplitude at age 10 weeks correlated significantly with clinical disease onset and survival. Electrophysiological tests increased their survival prediction accuracy when evaluated at later stages of the disease and also predicted the amount of lumbar spinal motoneuron preservation.

Conclusions: electrophysiological tests predict clinical disease onset, survival, and spinal motoneuron preservation in SOD1(G93A) mice. This is a methodological improvement for preclinical studies
0148-639X
943-949
Mancuso, R.
05786562-a993-4e37-926e-3c1fcf50b36d
Osta, R.
86d1bc86-c43c-41e4-8fde-a1e0758ff737
Navarro, X.
e02f3576-d8d3-495f-ba77-37e4f8769974
Mancuso, R.
05786562-a993-4e37-926e-3c1fcf50b36d
Osta, R.
86d1bc86-c43c-41e4-8fde-a1e0758ff737
Navarro, X.
e02f3576-d8d3-495f-ba77-37e4f8769974

Mancuso, R., Osta, R. and Navarro, X. (2014) Pre-symptomatic electrophysiological tests predict clinical disease onset and survival in SOD1G93A ALS mice. Muscle & Nerve, 50 (6), 943-949. (doi:10.1002/mus.24237). (PMID:24619579 )

Record type: Article

Abstract

Introduction: we assessed the predictive value of electrophysiological tests as a marker of clinical disease onset and survival in superoxide-dismutase 1 (SOD1)(G93A) mice.

Methods: we evaluated the accuracy of electrophysiological tests in differentiating transgenic versus wild-type mice. We made a correlation analysis of electrophysiological parameters and the onset of symptoms, survival, and number of spinal motoneurons.

Results: presymptomatic electrophysiological tests show great accuracy in differentiating transgenic versus wild-type mice, with the most sensitive parameter being the tibialis anterior compound muscle action potential (CMAP) amplitude. The CMAP amplitude at age 10 weeks correlated significantly with clinical disease onset and survival. Electrophysiological tests increased their survival prediction accuracy when evaluated at later stages of the disease and also predicted the amount of lumbar spinal motoneuron preservation.

Conclusions: electrophysiological tests predict clinical disease onset, survival, and spinal motoneuron preservation in SOD1(G93A) mice. This is a methodological improvement for preclinical studies

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More information

e-pub ahead of print date: 30 October 2014
Published date: December 2014
Organisations: Centre for Biological Sciences

Identifiers

Local EPrints ID: 376097
URI: http://eprints.soton.ac.uk/id/eprint/376097
ISSN: 0148-639X
PURE UUID: d30be5a5-0bc9-4f17-8eb0-0f5ae4cadac8

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Date deposited: 24 Apr 2015 08:45
Last modified: 15 Jul 2019 21:23

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