title: Evolution of gait abnormalities in SOD1(G93A) transgenic mice.
abstract: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the loss of upper and lower motoneurons. Clinically, it is manifested by weakness, muscle atrophy and progressive paralysis and ends up with patients' death 2-5 years after diagnosis. Although these symptoms lead in many cases to gait deficits in patients, an exhaustive locomotor profile of animal models mimicking the disease has not been assessed yet. In this work we evaluated the locomotor performance of the SOD1(G93A) mouse model of ALS using computerized treadmill gait analysis. SOD1(G93A) mice presented early (8 weeks of age) gait abnormalities, evidenced by an increase in the time of the propulsion phase of hindlimb stance. The alterations progressed during the disease until a complete disturbance of normal gait. This finding is meaningful to the field because the identification of a significant difference in a functional endpoint as early as 8 weeks might be a step forward resolving the debate about treatment of mice prior to the symptomatic phase in efficacy studies. These results also point out that digitizing analysis of treadmill locomotion may be useful to evaluate whether new therapeutic approaches are improving functional outcome of the animals.
authors: 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: Renzo
authors: R
authors: Mancuso
authors: Sara
authors: S
authors: Oliván
authors: Rosario
authors: R
authors: Osta
authors: Xavier
authors: X
authors: Navarro
mesh_terms: D000704:Analysis of Variance; D000818:Animals; D018450:Disease Progression; D004576:Electromyography; D005121:Extremities / Q000503:physiopathology; D020233:Gait Disorders, Neurologic / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D008124:Locomotion / Q000235:genetics; D051379:Mice; D008822:Mice, Transgenic; D009043:Motor Activity / Q000235:genetics; D009431:Neural Conduction / Q000235:genetics; D011597:Psychomotor Performance / Q000502:physiology; D045442:Rotarod Performance Test; D012584:Sciatic Nerve / Q000503:physiopathology; D013482:Superoxide Dismutase / Q000235:genetics*; D013997:Time Factors; D014741:Video Recording
chemical_list: C109415:SOD1 G93A protein; D013482:Superoxide Dismutase