Tyrosyl-DNA phosphodiesterase 1 (Tdp1) is a DNA repair enzyme that can remove a variety of covalent adducts from DNA through hydrolysis of a 3'-phosphodiester bond, giving rise to DNA with a free 3' phosphate [, ]. Mutations in human TDP1 gene cause spinocerebellar ataxia, autosomal recessive, with axonal neuropathy (SCAN1), which is a clinically and genetically heterogeneous group of cerebellar disorders [].
