This potential danger to the cell is highlighted by the catalytic human Tdp1 (H493R) mutant, which is the molecular basis for the rare autosomal recessive neurodegenerative disease spinocerebellar ataxia with axonal neuropathy (SCAN1)[29,33]. This evidence concerns the gene TDP1 and spinocerebellar ataxia, autosomal recessive, with axonal neuropathy 1.