{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T11:33:46Z","timestamp":1789558426117,"version":"build-2803163510"},"reference-count":64,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2020,2,10]],"date-time":"2020-02-10T00:00:00Z","timestamp":1581292800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["1144806"],"award-info":[{"award-number":["1144806"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["1021005"],"award-info":[{"award-number":["1021005"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,4,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Patients with ataxia-telangiectasia (A-T) lack a functional ATM kinase protein and exhibit defective repair of DNA double-stranded breaks and response to oxidative stress. We show that CRISPR\/Cas9-assisted gene correction combined with piggyBac (PB) transposon-mediated excision of the selection cassette enables seamless restoration of functional ATM alleles in induced pluripotent stem cells from an A-T patient carrying compound heterozygous exonic missense\/frameshift mutations, and from a patient with a homozygous splicing acceptor mutation of an internal coding exon. We show that the correction of one allele restores expression of ~\u200950% of full-length ATM protein and ameliorates DNA damage-induced activation (auto-phosphorylation) of ATM and phosphorylation of its downstream targets, KAP-1 and H2AX. Restoration of ATM function also normalizes radiosensitivity, mitochondrial ROS production and oxidative-stress-induced apoptosis levels in A-T iPSC lines, demonstrating that restoration of a single ATM allele is sufficient to rescue key ATM functions. Our data further show that despite the absence of a functional ATM kinase, homology-directed repair and seamless correction of a pathogenic ATM mutation is possible. The isogenic pairs of A-T and gene-corrected iPSCs described here constitute valuable tools for elucidating the role of ATM in ageing and A-T pathogenesis.<\/jats:p>","DOI":"10.1093\/hmg\/ddaa023","type":"journal-article","created":{"date-parts":[[2020,2,5]],"date-time":"2020-02-05T07:10:15Z","timestamp":1580886615000},"page":"990-1001","source":"Crossref","is-referenced-by-count":20,"title":["Correction of ATM mutations in iPS cells from two ataxia-telangiectasia patients restores DNA damage and oxidative stress responses"],"prefix":"10.1093","volume":"29","author":[{"given":"Dmitry A","family":"Ovchinnikov","sequence":"first","affiliation":[{"name":"Australian Institute for Bioengineering and Nanotechnology (AIBN), The University of Queensland, St. Lucia, Brisbane, QLD 4072, Australia"},{"name":"StemCore, The University of Queensland, St. Lucia, Brisbane, QLD 4072, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah L","family":"Withey","sequence":"first","affiliation":[{"name":"Australian Institute for Bioengineering and Nanotechnology (AIBN), The University of Queensland, St. Lucia, Brisbane, QLD 4072, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannah C","family":"Leeson","sequence":"first","affiliation":[{"name":"Australian Institute for Bioengineering and Nanotechnology (AIBN), The University of Queensland, St. Lucia, Brisbane, QLD 4072, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"U Wang","family":"Lei","sequence":"first","affiliation":[{"name":"Australian Institute for Bioengineering and Nanotechnology (AIBN), The University of Queensland, St. Lucia, Brisbane, QLD 4072, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashmitha","family":"Sundarrajan","sequence":"first","affiliation":[{"name":"Australian Institute for Bioengineering and Nanotechnology (AIBN), The University of Queensland, St. Lucia, Brisbane, QLD 4072, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keerat","family":"Junday","sequence":"first","affiliation":[{"name":"Australian Institute for Bioengineering and Nanotechnology (AIBN), The University of Queensland, St. Lucia, Brisbane, QLD 4072, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Pewarchuk","sequence":"first","affiliation":[{"name":"Australian Institute for Bioengineering and Nanotechnology (AIBN), The University of Queensland, St. Lucia, Brisbane, QLD 4072, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abrey J","family":"Yeo","sequence":"first","affiliation":[{"name":"UQ Centre for Clinical Research (UQCCR), The University of Queensland, Herston, Brisbane, QLD 4006, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda W","family":"Kijas","sequence":"first","affiliation":[{"name":"Australian Institute for Bioengineering and Nanotechnology (AIBN), The University of Queensland, St. Lucia, Brisbane, QLD 4072, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin F","family":"Lavin","sequence":"first","affiliation":[{"name":"UQ Centre for Clinical Research (UQCCR), The University of Queensland, Herston, Brisbane, QLD 4006, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ernst J","family":"Wolvetang","sequence":"first","affiliation":[{"name":"Australian Institute for Bioengineering and Nanotechnology (AIBN), The University of Queensland, St. Lucia, Brisbane, QLD 4072, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,2,10]]},"reference":[{"key":"2020041507553133200_ref1","doi-asserted-by":"crossref","first-page":"1674","DOI":"10.1126\/science.281.5383.1674","article-title":"Enhanced phosphorylation of p53 by ATM in response to DNA damage","volume":"281","author":"Banin","year":"1998","journal-title":"Science"},{"key":"2020041507553133200_ref2","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1038\/nature01368","article-title":"DNA damage activates ATM through intermolecular autophosphorylation and dimer dissociation","volume":"421","author":"Bakkenist","year":"2003","journal-title":"Nature"},{"key":"2020041507553133200_ref3","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1038\/nrm2514","article-title":"Ataxia-telangiectasia: from a rare disorder to a paradigm for cell signalling and cancer","volume":"9","author":"Lavin","year":"2008","journal-title":"Nat. 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Care Med."},{"key":"2020041507553133200_ref41","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1038\/sj.cdd.4400476","article-title":"Emerging roles of caspase-3 in apoptosis","volume":"6","author":"Porter","year":"1999","journal-title":"Cell Death Differ."},{"key":"2020041507553133200_ref42","doi-asserted-by":"crossref","first-page":"546","DOI":"10.1038\/emboj.2010.330","article-title":"ATM activates the pentose phosphate pathway promoting anti-oxidant defence and DNA repair","volume":"30","author":"Cosentino","year":"2011","journal-title":"EMBO J."},{"key":"2020041507553133200_ref43","doi-asserted-by":"crossref","first-page":"4029","DOI":"10.1074\/jbc.M410344200","article-title":"Full activation of PKB\/Akt in response to insulin or ionizing radiation is mediated through ATM","volume":"280","author":"Viniegra","year":"2005","journal-title":"J. Biol. Chem."},{"key":"2020041507553133200_ref44","doi-asserted-by":"crossref","first-page":"1555","DOI":"10.1016\/j.cellsig.2008.04.011","article-title":"ATM protein kinase mediates full activation of Akt and regulates glucose transporter 4 translocation by insulin in muscle cells","volume":"20","author":"Halaby","year":"2008","journal-title":"Cell. Signal."},{"key":"2020041507553133200_ref45","doi-asserted-by":"crossref","first-page":"5787","DOI":"10.1128\/MCB.00347-10","article-title":"Requirement of the ATM\/p53 tumor suppressor pathway for glucose homeostasis","volume":"30","author":"Armata","year":"2010","journal-title":"Mol. Cell. 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Natl. Cancer Inst."},{"key":"2020041507553133200_ref50","doi-asserted-by":"crossref","first-page":"S177","DOI":"10.1080\/09553009414552011","article-title":"Cancer risks in A-T heterozygotes","volume":"66","author":"Easton","year":"1994","journal-title":"Int. J. Radiat. Biol."},{"key":"2020041507553133200_ref51","first-page":"1938","article-title":"ATM protein and p53-serine 15 phosphorylation in ataxia-telangiectasia (AT) patients and at heterozygotes","volume":"82","author":"Delia","year":"2000","journal-title":"Br. J. Cancer"},{"key":"2020041507553133200_ref52","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1667\/RR3604.1","article-title":"Levels of gamma-H2AX foci after low-dose-rate irradiation reveal a DNA DSB rejoining defect in cells from human ATM heterozygotes in two at families and in another apparently normal individual","volume":"166","author":"Kato","year":"2006","journal-title":"Radiat. 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