[HTML][HTML] Alsin, the product of ALS2 gene, suppresses SOD1 mutant neurotoxicity through RhoGEF domain by interacting with SOD1 mutants

K Kanekura, Y Hashimoto, T Niikura, S Aiso… - Journal of Biological …, 2004 - ASBMB
Mutation of the ALS2 gene encoding alsin is linked to the onset of autosomal recessive
motor neuron diseases, including juvenile-onset amyotrophic lateral sclerosis (ALS). Alsin
long form (LF) belongs to the family of the guanine nucleotide exchanging factor (GEF) for
small GTPases. Expression of alsin LF, but not alsin short form, protected motor neuronal
cells from toxicity induced by mutants of the Cu/Zn-superoxide dismutase (SOD1) gene,
which cause autosomal dominant ALS. In contrast, expression of alsin did not suppress …