tyrosyl-DNA phosphodiesterase 1; tyr-DNA phosphodiesterase 1 (TDP1)

Function: 1) repair of covalent topoisomerase 1-DNA complexes 2) modulation of topological changes induced during DNA replication & transcription 3) efficient repair of stalled toposomerase 1-DNA complexes - catalyzes the hydrolysis of the phosphodiester bond between Tyr & DNA 3'phosphate 4) repair of free-radical mediated DNA double-strand breaks - removes glycolate from single-stranded DNA containing a 3'-phosphoglycolate Structure: - monomer - consists of 2 similar domains that are related by a pseudo-2-fold axis of symmetry Compartment: nuclear Expression: - ubiquitously expressed - similar expression throughout the central nervous system Pathology: - defects in TDP1 are the cause of: spinocerebellar ataxia with axonal neuropathy (SCAN1)

Related

spinocerebellar ataxia with axonal neuropathy (SCAN1) topoisomerase-1 (TOP1)

General

nuclear protein phosphodiesterase

Properties

SIZE: entity length = 608 aa MW = 68 kD COMPARTMENT: cytoplasm cell nucleus MOTIF: Thr phosphorylation site {T11} Ser phosphorylation site {S13} Ser phosphorylation site {S14} Ser phosphorylation site {S15} Ser phosphorylation site {S61} Thr phosphorylation site {T147} Ser phosphorylation site {S148} histidine residue {H263} binding site SITE: 265-265 FOR-BINDING-OF: Substrate DNA interaction {400-403} histidine residue {H493} binding site SITE: 495-495 FOR-BINDING-OF: Substrate serine residue {518}

Database Correlations

OMIM correlations MORBIDMAP 607198 UniProt Q9NUW8 Pfam PF06087 Entrez Gene 55775 Kegg hsa:55775