title: Presymptomatic electrophysiological tests predict clinical onset and survival in SOD1(G93A) ALS mice.
abstract: BACKGROUND
  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.
authors.affiliation: Group of Neuroplasticity and Regeneration, Institute of Neurosciences and Department of Cell Biology, Physiology and Immunology, Universitat Autònoma de Barcelona, and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Bellaterra, Spain.
authors.forename: Renzo
authors.initials: R
authors.lastname: Mancuso
authors.forename: Rosario
authors.initials: R
authors.lastname: Osta
authors.forename: Xavier
authors.initials: X
authors.lastname: Navarro
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000401:mortality* / Q000503:physiopathology*; D000818:Animals; D004195:Disease Models, Animal; D018450:Disease Progression; D004594:Electrophysiology; D005071:Evoked Potentials / Q000502:physiology; D005260:Female; D008297:Male; D051379:Mice; D008817:Mice, Mutant Strains; D008822:Mice, Transgenic; D009046:Motor Neurons / Q000502:physiology*; D009431:Neural Conduction / Q000502:physiology; D011237:Predictive Value of Tests; D013482:Superoxide Dismutase / Q000235:genetics*; D000072105:Superoxide Dismutase-1; D015996:Survival Rate
chemical_list: C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1
keywords: SOD1; amyotrophic lateral sclerosis; biomarkers; electrophysiology; motoneuron disease