Amyotrophic lateral sclerosis (ALS)

Mouse models of amyotrophic lateral sclerosis (ALS) carry human mutations that cause ALS in patients, typically mutations in the SOD1 gene.

Amyotrophic lateral sclerosis pathology

amyotrophic-lateral-sclerosisALS is a progressive neurodegenerative disorder characterized by degeneration of motor neurons, and is associated with both motor and cognitive symptoms. Pathological mechanisms thought to be involved in the disease include protein aggregation and oxidative stress. Superoxide dismutase 1 (SOD1) was the first gene found to be associated with ALS, and subsequently evidence has been provided for the presence of SOD1 protein aggregates and increased oxidative stress as a result of mutations in the SOD1 gene, which codes for an antioxidant protein.

Behavioural readouts for ALS mouse models

Mouse models of ALS develop significant motor problems in our tests of motor function (e.g. Activity in an automated home-cage, Grip strength, Rotarod).