Search: onr:"swepub:oai:gup.ub.gu.se/264325" > Topoisomerase 3α Is...
Fältnamn | Indikatorer | Metadata |
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000 | 05657naa a2200769 4500 | |
001 | oai:gup.ub.gu.se/264325 | |
003 | SwePub | |
008 | 240528s2018 | |||||||||||000 ||eng| | |
009 | oai:prod.swepub.kib.ki.se:137407931 | |
024 | 7 | a https://gup.ub.gu.se/publication/2643252 URI |
024 | 7 | a https://doi.org/10.1016/j.molcel.2017.11.0332 DOI |
024 | 7 | a http://kipublications.ki.se/Default.aspx?queryparsed=id:1374079312 URI |
040 | a (SwePub)gud (SwePub)ki | |
041 | a eng | |
042 | 9 SwePub | |
072 | 7 | a ref2 swepub-contenttype |
072 | 7 | a art2 swepub-publicationtype |
100 | 1 | a Nicholls, Thomas J.u Gothenburg University,Göteborgs universitet,Institutionen för biomedicin, avdelningen för medicinsk kemi och cellbiologi,Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology4 aut |
245 | 1 0 | a Topoisomerase 3α Is Required for Decatenation and Segregation of Human mtDNA |
264 | 1 | b Elsevier BV,c 2018 |
520 | a How mtDNA replication is terminated and the newly formed genomes are separated remain unknown. We here demonstrate that the mitochondrial isoform of topoisomerase 3α (Top3α) fulfills this function, acting independently of its nuclear role as a component of the Holliday junction-resolving BLM-Top3α-RMI1-RMI2 (BTR) complex. Our data indicate that mtDNA replication termination occurs via a hemicatenane formed at the origin of H-strand replication and that Top3α is essential for resolving this structure. Decatenation is a prerequisite for separation of the segregating unit of mtDNA, the nucleoid, within the mitochondrial network. The importance of this process is highlighted in a patient with mitochondrial disease caused by biallelic pathogenic variants in TOP3A, characterized by muscle-restricted mtDNA deletions and chronic progressive external ophthalmoplegia (CPEO) plus syndrome. Our work establishes Top3α as an essential component of the mtDNA replication machinery and as the first component of the mtDNA separation machinery. Nicholls et al. identify a role for topoisomerase 3α in the separation of mtDNA following replication. Loss of Top3α activity impairs mtDNA segregation and, consequently, segregation of the mtDNA nucleoid within the mitochondrial network. Mutations in TOP3A cause human mitochondrial disease associated with mtDNA deletions and impaired mtDNA separation. © 2017 Elsevier Inc. | |
650 | 7 | a MEDICIN OCH HÄLSOVETENSKAPx Medicinska och farmaceutiska grundvetenskaperx Cell- och molekylärbiologi0 (SwePub)301082 hsv//swe |
650 | 7 | a MEDICAL AND HEALTH SCIENCESx Basic Medicinex Cell and Molecular Biology0 (SwePub)301082 hsv//eng |
650 | 7 | a NATURVETENSKAPx Biologix Cellbiologi0 (SwePub)106042 hsv//swe |
650 | 7 | a NATURAL SCIENCESx Biological Sciencesx Cell Biology0 (SwePub)106042 hsv//eng |
653 | a DNA replication | |
653 | a DNA separation | |
653 | a mitochondrion | |
653 | a nucleoid | |
653 | a progressive external ophthalmoplegia | |
653 | a segregation | |
653 | a topoisomerase | |
653 | a catenane | |
653 | a DNA topoisomerase | |
653 | a mitochondrial DNA | |
653 | a allelism | |
653 | a Article | |
653 | a chronic progressive external ophthalmoplegia | |
653 | a complex formation | |
653 | a controlled study | |
653 | a DNA determination | |
653 | a DNA structure | |
653 | a gene deletion | |
653 | a genetic variation | |
653 | a human | |
653 | a human cell | |
653 | a protein function | |
700 | 1 | a Nadalutti, C. A.4 aut |
700 | 1 | a Motori, E.4 aut |
700 | 1 | a Sommerville, E. W.4 aut |
700 | 1 | a Gorman, G. S.4 aut |
700 | 1 | a Basu, Swaraju Gothenburg University,Göteborgs universitet,Institutionen för biomedicin, avdelningen för medicinsk kemi och cellbiologi,Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology4 aut0 (Swepub:gu)xbasus |
700 | 1 | a Hoberg, Emily,d 1986u Gothenburg University,Göteborgs universitet,Institutionen för biomedicin, avdelningen för medicinsk kemi och cellbiologi,Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology4 aut0 (Swepub:gu)xhogem |
700 | 1 | a Turnbull, D. M.4 aut |
700 | 1 | a Chinnery, P. F.4 aut |
700 | 1 | a Larsson, N. G.u Karolinska Institutet4 aut |
700 | 1 | a Larsson, Erik,d 1975u Gothenburg University,Göteborgs universitet,Institutionen för biomedicin, avdelningen för medicinsk kemi och cellbiologi,Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology4 aut0 (Swepub:gu)xlarer |
700 | 1 | a Falkenberg, Maria,d 1968u Gothenburg University,Göteborgs universitet,Institutionen för biomedicin, avdelningen för medicinsk kemi och cellbiologi,Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology4 aut0 (Swepub:gu)xfamar |
700 | 1 | a Taylor, R. W.4 aut |
700 | 1 | a Griffith, J. D.4 aut |
700 | 1 | a Gustafsson, Claes M,d 1966u Gothenburg University,Göteborgs universitet,Institutionen för biomedicin, avdelningen för medicinsk kemi och cellbiologi,Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology4 aut0 (Swepub:gu)xgucla |
710 | 2 | a Göteborgs universitetb Institutionen för biomedicin, avdelningen för medicinsk kemi och cellbiologi4 org |
773 | 0 | t Molecular Celld : Elsevier BVg 69:1q 69:1x 1097-2765x 1097-4164 |
856 | 4 | u http://www.cell.com/article/S1097276517308924/pdf |
856 | 4 8 | u https://gup.ub.gu.se/publication/264325 |
856 | 4 8 | u https://doi.org/10.1016/j.molcel.2017.11.033 |
856 | 4 8 | u http://kipublications.ki.se/Default.aspx?queryparsed=id:137407931 |
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