HMGB1 and spinocerebellar ataxia type 1: Interestingly though, evidence from studies on spinocerebellar ataxia type 1 (SCA1)—a rare autosomal dominant neurodegenerative disease—showed that the reduction in HMGB1 expression was detrimental in the SCA1 mouse model, while its overexpression restored DNA damage in affected cerebellar Purkinje cells and spinal cord motor neurons, improving motor function and prolonging the life of SCA1 mice [17].