title: Effect of genetic background on onset and disease progression in the SOD1-G93A model of amyotrophic lateral sclerosis.
abstract: Knowledge of the potential effect of genetic background in disease models is important. The SOD1-G93A transgenic mouse is the most widely used model in amyotrophic lateral sclerosis (ALS). Since these animals show considerable variability both in the onset and the progression of the disease, this study aimed to characterize the potential differences between the two most widely used strains, C56BL/6 (B6) and B6SJL. A rotarod test was carried out to assess strength and motor coordination, while electrophysiology tests were performed to evaluate the function of upper and lower motor neurons. Survival of the animals and motor neuron loss were also studied. The results did not show any background effect regarding the rotarod test, despite the differences in the pattern of decline in central and peripheral motor conduction. The onset of motor neuron abnormalities was later in B6SJL mice, but progressed more rapidly. Lifespan was longer for B6 than for B6SJL animals. In conclusion, background differences in disease onset and progression are important. The characteristics of the strain should be taken into account in experimental design of therapeutic studies.
authors.affiliation: Group of Neuroplasticity and Regeneration, Institute of Neurosciences and Department of Cell Biology, Physiology and Immunology, Universitat Autònoma de Barcelona, Bellaterra.
authors.forename: Renzo
authors.initials: R
authors.lastname: Mancuso
authors.forename: Sara
authors.initials: S
authors.lastname: Oliván
authors.forename: Pilar
authors.initials: P
authors.lastname: Mancera
authors.forename: Andrea
authors.initials: A
authors.lastname: Pastén-Zamorano
authors.forename: Raquel
authors.initials: R
authors.lastname: Manzano
authors.forename: Caty
authors.initials: C
authors.lastname: Casas
authors.forename: Rosario
authors.initials: R
authors.lastname: Osta
authors.forename: Xavier
authors.initials: X
authors.lastname: Navarro
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology*; D000818:Animals; D004195:Disease Models, Animal; D018450:Disease Progression; D019054:Evoked Potentials, Motor; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D013482:Superoxide Dismutase / Q000235:genetics* / Q000378:metabolism; D016019:Survival Analysis
chemical_list: C109415:SOD1 G93A protein; D013482:Superoxide Dismutase