{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T03:58:58Z","timestamp":1792641538679,"version":"4.5.0"},"reference-count":28,"publisher":"National Academy of Sciences","issue":"23","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[1996,11,12]]},"abstract":"<jats:p>\n                    We have generated a mouse model for ataxia-telangiectasia by using\n gene targeting to generate mice that do not express the Atm protein.\n Atm-deficient mice are retarded in growth, do not produce mature sperm,\n and exhibit severe defects in T cell maturation while going on to\n develop thymomas. Atm-deficient fibroblasts grow poorly in culture and\n display a high level of double-stranded chromosome breaks.\n Atm-deficient thymocytes undergo spontaneous apoptosis\n                    <jats:italic>in vitro<\/jats:italic>\n                    significantly more than controls. Atm-deficient\n mice then exhibit many of the same symptoms found in\n ataxia-telangiectasia patients and in cells derived from them.\n Furthermore, we demonstrate that the Atm protein exists as two discrete\n molecular species, and that loss of one or of both of these can lead to\n the development of the disease.\n                  <\/jats:p>","DOI":"10.1073\/pnas.93.23.13084","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T10:32:33Z","timestamp":1027679553000},"page":"13084-13089","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":433,"title":["Pleiotropic defects in ataxia-telangiectasia protein-deficient\u2009mice"],"prefix":"10.1073","volume":"93","author":[{"given":"Ari","family":"Elson","sequence":"first","affiliation":[{"name":"Department of Genetics, Howard Hughes Medical Institute, Harvard\r Medical School, Boston, MA 02115; and Department of Pathology,\r Brigham and Women\u2019s Hospital, Harvard Medical School, Boston, MA 02115"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yaoqi","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Genetics, Howard Hughes Medical Institute, Harvard\r Medical School, Boston, MA 02115; and Department of Pathology,\r Brigham and Women\u2019s Hospital, Harvard Medical School, Boston, MA 02115"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cathie\u2009J.","family":"Daugherty","sequence":"additional","affiliation":[{"name":"Department of Genetics, Howard Hughes Medical Institute, Harvard\r Medical School, Boston, MA 02115; and Department of Pathology,\r Brigham and Women\u2019s Hospital, Harvard Medical School, Boston, MA 02115"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cynthia\u2009C.","family":"Morton","sequence":"additional","affiliation":[{"name":"Department of Genetics, Howard Hughes Medical Institute, Harvard\r Medical School, Boston, MA 02115; and Department of Pathology,\r Brigham and Women\u2019s Hospital, Harvard Medical School, Boston, MA 02115"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fen","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Genetics, Howard Hughes Medical Institute, Harvard\r Medical School, Boston, MA 02115; and Department of Pathology,\r Brigham and Women\u2019s Hospital, Harvard Medical School, Boston, MA 02115"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juanita","family":"Campos-Torres","sequence":"additional","affiliation":[{"name":"Department of Genetics, Howard Hughes Medical Institute, Harvard\r Medical School, Boston, MA 02115; and Department of Pathology,\r Brigham and Women\u2019s Hospital, Harvard Medical School, Boston, MA 02115"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Leder","sequence":"additional","affiliation":[{"name":"Department of Genetics, Howard Hughes Medical Institute, Harvard\r Medical School, Boston, MA 02115; and Department of Pathology,\r Brigham and Women\u2019s Hospital, Harvard Medical School, Boston, MA 02115"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[1996,11,12]]},"reference":[{"key":"e_1_3_2_1_2","first-page":"347","volume-title":"Handbook of Clinical Neurology","author":"Sedgewick R","year":"1991","unstructured":"R Sedgewick, E Boder Handbook of Clinical Neurology, eds P Vinken, G Bruyn, H Klawans (Elsevier, New York), pp. 347\u2013423 (1991)."},{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198705213162101"},{"key":"e_1_3_2_3_2","doi-asserted-by":"crossref","unstructured":"Easton D.\u2009F. 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