InterPro domain: IPR010347

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 [ 1 , 2 ].

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 [ 3 ].

1. Conversion of phosphoglycolate to phosphate termini on 3' overhangs of DNA double strand breaks by the human tyrosyl-DNA phosphodiesterase hTdp1. J. Biol. Chem. 277, 27162-8
2. Tyrosyl-DNA phosphodiesterase and the repair of 3'-phosphoglycolate-terminated DNA double-strand breaks. DNA Repair (Amst.) 8, 901-11
3. Human Tdp1 cleaves a broad spectrum of substrates, including phosphoamide linkages. J. Biol. Chem. 280, 36518-28