{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T20:12:16Z","timestamp":1791231136497,"version":"4.1.0"},"reference-count":36,"publisher":"Springer Science and Business Media LLC","issue":"11","license":[{"start":{"date-parts":[[2011,10,9]],"date-time":"2011-10-09T00:00:00Z","timestamp":1318118400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nat Struct Mol Biol"],"published-print":{"date-parts":[[2011,11]]},"DOI":"10.1038\/nsmb.2146","type":"journal-article","created":{"date-parts":[[2011,10,10]],"date-time":"2011-10-10T15:09:55Z","timestamp":1318259395000},"page":"1189-1195","source":"Crossref","is-referenced-by-count":36,"title":["Structure of an aprataxin\u2013DNA complex with insights into AOA1 neurodegenerative disease"],"prefix":"10.1038","volume":"18","author":[{"given":"Percy","family":"Tumbale","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C Denise","family":"Appel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolf","family":"Kraehenbuehl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick D","family":"Robertson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica S","family":"Williams","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joe","family":"Krahn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivan","family":"Ahel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R Scott","family":"Williams","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2011,10,9]]},"reference":[{"key":"BFnsmb2146_CR1","doi-asserted-by":"publisher","first-page":"473","DOI":"10.1038\/nature03082","volume":"432","author":"JM Pascal","year":"2004","unstructured":"Pascal, J.M., O'Brien, P.J., Tomkinson, A.E. & Ellenberger, T. Human DNA ligase I completely encircles and partially unwinds nicked DNA. Nature 432, 473\u2013478 (2004).","journal-title":"Nature"},{"key":"BFnsmb2146_CR2","doi-asserted-by":"publisher","first-page":"313","DOI":"10.1146\/annurev.biochem.77.061306.123941","volume":"77","author":"T Ellenberger","year":"2008","unstructured":"Ellenberger, T. & Tomkinson, A.E. Eukaryotic DNA ligases: structural and functional insights. Annu. Rev. Biochem. 77, 313\u2013338 (2008).","journal-title":"Annu. Rev. Biochem."},{"key":"BFnsmb2146_CR3","doi-asserted-by":"publisher","first-page":"713","DOI":"10.1038\/nature05164","volume":"443","author":"I Ahel","year":"2006","unstructured":"Ahel, I. et al. The neurodegenerative disease protein aprataxin resolves abortive DNA ligation intermediates. Nature 443, 713\u2013716 (2006).","journal-title":"Nature"},{"key":"BFnsmb2146_CR4","doi-asserted-by":"publisher","first-page":"1354","DOI":"10.1128\/MCB.01471-08","volume":"29","author":"JJ Reynolds","year":"2009","unstructured":"Reynolds, J.J. et al. Defective DNA ligation during short-patch single-strand break repair in ataxia oculomotor apraxia 1. Mol. Cell. Biol. 29, 1354\u20131362 (2009).","journal-title":"Mol. Cell. Biol."},{"key":"BFnsmb2146_CR5","doi-asserted-by":"publisher","first-page":"4102","DOI":"10.1093\/hmg\/ddp359","volume":"18","author":"JL Harris","year":"2009","unstructured":"Harris, J.L. et al. Aprataxin, poly-ADP ribose polymerase 1 (PARP-1) and apurinic endonuclease 1 (APE1) function together to protect the genome against oxidative damage. Hum. Mol. Genet. 18, 4102\u20134117 (2009).","journal-title":"Hum. Mol. Genet."},{"key":"BFnsmb2146_CR6","doi-asserted-by":"publisher","first-page":"184","DOI":"10.1038\/ng1001-184","volume":"29","author":"H Date","year":"2001","unstructured":"Date, H. et al. Early-onset ataxia with ocular motor apraxia and hypoalbuminemia is caused by mutations in a new HIT superfamily gene. Nat. Genet. 29, 184\u2013188 (2001).","journal-title":"Nat. Genet."},{"key":"BFnsmb2146_CR7","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1038\/ng1001-189","volume":"29","author":"MC Moreira","year":"2001","unstructured":"Moreira, M.C. et al. The gene mutated in ataxia-ocular apraxia 1 encodes the new HIT\/Zn-finger protein aprataxin. Nat. Genet. 29, 189\u2013193 (2001).","journal-title":"Nat. Genet."},{"key":"BFnsmb2146_CR8","doi-asserted-by":"publisher","first-page":"539","DOI":"10.1212\/01.WNL.0000150588.75281.58","volume":"64","author":"CM Quinzii","year":"2005","unstructured":"Quinzii, C.M. et al. Coenzyme Q deficiency and cerebellar ataxia associated with an aprataxin mutation. Neurology 64, 539\u2013541 (2005).","journal-title":"Neurology"},{"key":"BFnsmb2146_CR9","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1016\/j.parkreldis.2006.08.010","volume":"13","author":"Y Baba","year":"2007","unstructured":"Baba, Y. et al. Aprataxin (APTX) gene mutations resembling multiple system atrophy. Parkinsonism Relat. Disord. 13, 139\u2013142 (2007).","journal-title":"Parkinsonism Relat. Disord."},{"key":"BFnsmb2146_CR10","doi-asserted-by":"publisher","first-page":"162","DOI":"10.1002\/ana.21078","volume":"61","author":"M Hirano","year":"2007","unstructured":"Hirano, M. et al. DNA single-strand break repair is impaired in aprataxin-related ataxia. Ann. Neurol. 61, 162\u2013174 (2007).","journal-title":"Ann. Neurol."},{"key":"BFnsmb2146_CR11","doi-asserted-by":"publisher","first-page":"485","DOI":"10.1007\/s00018-004-4441-0","volume":"62","author":"P Mosesso","year":"2005","unstructured":"Mosesso, P. et al. The novel human gene aprataxin is directly involved in DNA single-strand-break repair. Cell. Mol. Life Sci. 62, 485\u2013491 (2005).","journal-title":"Cell. Mol. Life Sci."},{"key":"BFnsmb2146_CR12","doi-asserted-by":"publisher","first-page":"690","DOI":"10.1016\/j.dnarep.2010.03.006","volume":"9","author":"JM Daley","year":"2010","unstructured":"Daley, J.M., Wilson, T.E. & Ramotar, D. Genetic interactions between HNT3\/Aprataxin and RAD27\/FEN1 suggest parallel pathways for 5\u2032 end processing during base excision repair. DNA Repair (Amst.) 9, 690\u2013699 (2010).","journal-title":"DNA Repair (Amst.)"},{"key":"BFnsmb2146_CR13","doi-asserted-by":"publisher","first-page":"672","DOI":"10.1016\/j.dnarep.2009.01.016","volume":"8","author":"GP Deshpande","year":"2009","unstructured":"Deshpande, G.P. et al. Screening a genome-wide S. pombe deletion library identifies novel genes and pathways involved in genome stability maintenance. DNA Repair (Amst.) 8, 672\u2013679 (2009).","journal-title":"DNA Repair (Amst.)"},{"key":"BFnsmb2146_CR14","doi-asserted-by":"publisher","first-page":"619","DOI":"10.1038\/nrg2380","volume":"9","author":"KW Caldecott","year":"2008","unstructured":"Caldecott, K.W. Single-strand break repair and genetic disease. Nat. Rev. Genet. 9, 619\u2013631 (2008).","journal-title":"Nat. Rev. Genet."},{"key":"BFnsmb2146_CR15","doi-asserted-by":"publisher","first-page":"9469","DOI":"10.1074\/jbc.M611489200","volume":"282","author":"U Rass","year":"2007","unstructured":"Rass, U., Ahel, I. & West, S.C. Actions of aprataxin in multiple DNA repair pathways. J. Biol. Chem. 282, 9469\u20139474 (2007).","journal-title":"J. Biol. Chem."},{"key":"BFnsmb2146_CR16","doi-asserted-by":"publisher","first-page":"286","DOI":"10.1126\/science.278.5336.286","volume":"278","author":"CD Lima","year":"1997","unstructured":"Lima, C.D., Klein, M.G. & Hendrickson, W.A. Structure-based analysis of catalysis and substrate definition in the HIT protein family. Science 278, 286\u2013290 (1997).","journal-title":"Science"},{"key":"BFnsmb2146_CR17","doi-asserted-by":"publisher","first-page":"9003","DOI":"10.1021\/bi025942q","volume":"41","author":"C Brenner","year":"2002","unstructured":"Brenner, C. Hint, Fhit, and GalT: function, structure, evolution, and mechanism of three branches of the histidine triad superfamily of nucleotide hydrolases and transferases. Biochemistry 41, 9003\u20139014 (2002).","journal-title":"Biochemistry"},{"key":"BFnsmb2146_CR18","doi-asserted-by":"publisher","first-page":"33994","DOI":"10.1074\/jbc.M807124200","volume":"283","author":"U Rass","year":"2008","unstructured":"Rass, U., Ahel, I. & West, S.C. Molecular mechanism of DNA deadenylation by the neurological disease protein aprataxin. J. Biol. Chem. 283, 33994\u201334001 (2008).","journal-title":"J. Biol. Chem."},{"key":"BFnsmb2146_CR19","doi-asserted-by":"publisher","first-page":"1493","DOI":"10.1016\/j.dnarep.2004.06.017","volume":"3","author":"PM Clements","year":"2004","unstructured":"Clements, P.M. et al. The ataxia-oculomotor apraxia 1 gene product has a role distinct from ATM and interacts with the DNA strand break repair proteins XRCC1 and XRCC4. DNA Repair (Amst.) 3, 1493\u20131502 (2004).","journal-title":"DNA Repair (Amst.)"},{"key":"BFnsmb2146_CR20","doi-asserted-by":"publisher","first-page":"1489","DOI":"10.1093\/nar\/gkp1149","volume":"38","author":"OJ Becherel","year":"2010","unstructured":"Becherel, O.J. et al. CK2 phosphorylation-dependent interaction between aprataxin and MDC1 in the DNA damage response. Nucleic Acids Res. 38, 1489\u20131503 (2010).","journal-title":"Nucleic Acids Res."},{"key":"BFnsmb2146_CR21","doi-asserted-by":"publisher","first-page":"763","DOI":"10.1016\/S0969-2126(97)00231-1","volume":"5","author":"CD Lima","year":"1997","unstructured":"Lima, C.D. et al. MAD analysis of FHIT, a putative human tumor suppressor from the HIT protein family. Structure 5, 763\u2013774 (1997).","journal-title":"Structure"},{"key":"BFnsmb2146_CR22","doi-asserted-by":"publisher","first-page":"809","DOI":"10.1126\/science.2028256","volume":"252","author":"NP Pavletich","year":"1991","unstructured":"Pavletich, N.P. & Pabo, C.O. Zinc finger-DNA recognition: crystal structure of a Zif268-DNA complex at 2.1 A. Science 252, 809\u2013817 (1991).","journal-title":"Science"},{"key":"BFnsmb2146_CR23","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1146\/annurev-biochem-010909-095056","volume":"79","author":"A Klug","year":"2010","unstructured":"Klug, A. The discovery of zinc fingers and their applications in gene regulation and genome manipulation. Annu. Rev. Biochem. 79, 213\u2013231 (2010).","journal-title":"Annu. Rev. Biochem."},{"key":"BFnsmb2146_CR24","doi-asserted-by":"publisher","first-page":"3797","DOI":"10.1093\/nar\/gkm158","volume":"35","author":"T Takahashi","year":"2007","unstructured":"Takahashi, T. et al. Aprataxin, causative gene product for EAOH\/AOA1, repairs DNA single-strand breaks with damaged 3\u2032-phosphate and 3\u2032-phosphoglycolate ends. Nucleic Acids Res. 35, 3797\u20133809 (2007).","journal-title":"Nucleic Acids Res."},{"key":"BFnsmb2146_CR25","doi-asserted-by":"publisher","first-page":"13939","DOI":"10.1074\/jbc.M507946200","volume":"281","author":"AW Kijas","year":"2006","unstructured":"Kijas, A.W., Harris, J.L., Harris, J.M. & Lavin, M.F. Aprataxin forms a discrete branch in the HIT (histidine triad) superfamily of proteins with both DNA\/RNA binding and nucleotide hydrolase activities. J. Biol. Chem. 281, 13939\u201313948 (2006).","journal-title":"J. Biol. Chem."},{"key":"BFnsmb2146_CR26","doi-asserted-by":"publisher","first-page":"868","DOI":"10.1212\/01.WNL.0000048562.88536.A4","volume":"60","author":"C Tranchant","year":"2003","unstructured":"Tranchant, C., Fleury, M., Moreira, M.C., Koenig, M. & Warter, J.M. Phenotypic variability of aprataxin gene mutations. Neurology 60, 868\u2013870 (2003).","journal-title":"Neurology"},{"key":"BFnsmb2146_CR27","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1016\/j.mrfmmm.2005.03.012","volume":"577","author":"JL Huffman","year":"2005","unstructured":"Huffman, J.L., Sundheim, O. & Tainer, J.A. DNA base damage recognition and removal: new twists and grooves. Mutat. Res. 577, 55\u201376 (2005).","journal-title":"Mutat. Res."},{"key":"BFnsmb2146_CR28","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1038\/82818","volume":"7","author":"SH Wilson","year":"2000","unstructured":"Wilson, S.H. & Kunkel, T.A. Passing the baton in base excision repair. Nat. Struct. Biol. 7, 176\u2013178 (2000).","journal-title":"Nat. Struct. Biol."},{"key":"BFnsmb2146_CR29","doi-asserted-by":"publisher","first-page":"657","DOI":"10.1016\/j.molcel.2005.02.012","volume":"17","author":"NK Bernstein","year":"2005","unstructured":"Bernstein, N.K. et al. The molecular architecture of the mammalian DNA repair enzyme, polynucleotide kinase. Mol. Cell 17, 657\u2013670 (2005).","journal-title":"Mol. Cell"},{"key":"BFnsmb2146_CR30","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1017\/S0033583510000089","volume":"43","author":"A Klug","year":"2010","unstructured":"Klug, A. The discovery of zinc fingers and their development for practical applications in gene regulation and genome manipulation. Q. Rev. Biophys. 43, 1\u201321 (2010).","journal-title":"Q. Rev. Biophys."},{"key":"BFnsmb2146_CR31","doi-asserted-by":"publisher","first-page":"2375","DOI":"10.1158\/1078-0432.CCR-09-3275","volume":"16","author":"H Dopeso","year":"2010","unstructured":"Dopeso, H. et al. Aprataxin tumor levels predict response of colorectal cancer patients to irinotecan-based treatment. Clin. Cancer Res. 16, 2375\u20132382 (2010).","journal-title":"Clin. Cancer Res."},{"key":"BFnsmb2146_CR32","doi-asserted-by":"publisher","first-page":"307","DOI":"10.1016\/S0076-6879(97)76066-X","volume":"276","author":"Z Otwinowski","year":"1997","unstructured":"Otwinowski, Z. & Minor, W. Processing of X-ray diffraction data collected in oscillation mode. Methods Enzymol. 276, 307\u2013326 (1997).","journal-title":"Methods Enzymol."},{"key":"BFnsmb2146_CR33","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1107\/S0907444909052925","volume":"66","author":"PD Adams","year":"2010","unstructured":"Adams, P.D. et al. PHENIX: a comprehensive Python-based system for macromolecular structure solution. Acta Crystallogr. D Biol. Crystallogr. 66, 213\u2013221 (2010).","journal-title":"Acta Crystallogr. D Biol. Crystallogr."},{"key":"BFnsmb2146_CR34","first-page":"34","volume":"31","author":"K Cowtan","year":"1994","unstructured":"Cowtan, K. dm: an automated procedure for phase improvement by density modification. Joint CCP4 and ESF-EACBM Newslett. on Protein Crystallogr. 31, 34\u201338 (1994).","journal-title":"Joint CCP4 and ESF-EACBM Newslett. on Protein Crystallogr."},{"key":"BFnsmb2146_CR35","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1107\/S0108767390010224","volume":"47","author":"TA Jones","year":"1991","unstructured":"Jones, T.A., Zou, J.Y., Cowan, S.W. & Kjeldgaard, M. Improved methods for building protein models in electron density maps and the location of errors in these models. Acta Crystallogr. A 47, 110\u2013119 (1991).","journal-title":"Acta Crystallogr. A"},{"key":"BFnsmb2146_CR36","doi-asserted-by":"publisher","first-page":"240","DOI":"10.1107\/S0907444996012255","volume":"53","author":"GN Murshudov","year":"1997","unstructured":"Murshudov, G.N., Vagin, A.A. & Dodson, E.J. Refinement of macromolecular structures by the maximum-likelihood method. Acta Crystallogr. D Biol. Crystallogr. 53, 240\u2013255 (1997).","journal-title":"Acta Crystallogr. D Biol. Crystallogr."}],"container-title":["Nature Structural & Molecular Biology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.nature.com\/articles\/nsmb.2146.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/nsmb.2146","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/nsmb.2146.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,19]],"date-time":"2023-05-19T04:30:08Z","timestamp":1684470608000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/nsmb.2146"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,10,9]]},"references-count":36,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2011,11]]}},"alternative-id":["BFnsmb2146"],"URL":"https:\/\/doi.org\/10.1038\/nsmb.2146","relation":{},"ISSN":["1545-9993","1545-9985"],"issn-type":[{"value":"1545-9993","type":"print"},{"value":"1545-9985","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,10,9]]}}}