{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T21:01:07Z","timestamp":1790370067976,"version":"4.1.0"},"reference-count":22,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2013,9,11]],"date-time":"2013-09-11T00:00:00Z","timestamp":1378857600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2013,9,11]],"date-time":"2013-09-11T00:00:00Z","timestamp":1378857600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Aging Cell"],"published-print":{"date-parts":[[2013,12]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    Cockayne syndrome (\n                    <jats:styled-content style=\"fixed-case\">CS<\/jats:styled-content>\n                    ) is a rare autosomal recessive segmental progeria characterized by growth failure, lipodystrophy, neurological abnormalities, and photosensitivity, but without skin cancer predisposition. Cockayne syndrome life expectancy ranges from 5 to 16\u00a0years for the two most severe forms (types II and I, respectively). Mouse models of\n                    <jats:styled-content style=\"fixed-case\">CS<\/jats:styled-content>\n                    have thus far been of limited value due to either very mild phenotypes, or premature death during postnatal development prior to weaning. The cause of death in severe\n                    <jats:styled-content style=\"fixed-case\">CS<\/jats:styled-content>\n                    models is unknown, but has been attributed to extremely rapid aging. Here, we found that providing mutant pups with soft food from as late as postnatal day 14 allowed survival past weaning with high penetrance independent of dietary macronutrient balance in a novel\n                    <jats:styled-content style=\"fixed-case\">CS<\/jats:styled-content>\n                    model (\n                    <jats:italic>Csa<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    <jats:italic>| Xpa<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    <jats:italic>)<\/jats:italic>\n                    . Survival past weaning revealed a number of\n                    <jats:styled-content style=\"fixed-case\">CS<\/jats:styled-content>\n                    \u2010like symptoms including small size, progressive loss of adiposity, and neurological symptoms, with a maximum lifespan of 19\u00a0weeks. Our results caution against interpretation of death before weaning as premature aging, and at the same time provide a valuable new tool for understanding mechanisms of progressive\n                    <jats:styled-content style=\"fixed-case\">CS<\/jats:styled-content>\n                    \u2010related progeroid symptoms including lipodystrophy and neurodysfunction.\n                  <\/jats:p>","DOI":"10.1111\/acel.12142","type":"journal-article","created":{"date-parts":[[2013,7,29]],"date-time":"2013-07-29T11:01:27Z","timestamp":1375095687000},"page":"1144-1147","source":"Crossref","is-referenced-by-count":23,"title":["Lifespan extension by dietary intervention in a mouse model of\n                    <scp>C<\/scp>\n                    ockayne\n                    <scp>S<\/scp>\n                    yndrome uncouples early postnatal development from segmental progeria"],"prefix":"10.1111","volume":"12","author":[{"given":"Lear E.","family":"Brace","sequence":"first","affiliation":[{"name":"Department of Genetics and Complex Diseases Harvard School of Public Health 655 Huntington Ave Boston MA 02115 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah C.","family":"Vose","sequence":"additional","affiliation":[{"name":"Department of Genetics and Complex Diseases Harvard School of Public Health 655 Huntington Ave Boston MA 02115 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorathy F.","family":"Vargas","sequence":"additional","affiliation":[{"name":"Department of Genetics and Complex Diseases Harvard School of Public Health 655 Huntington Ave Boston MA 02115 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuangyun","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Developmental Biology Harvard School of Dental Medicine 188 Longwood Avenue Boston MA 02115 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiu\u2010Ping","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Developmental Biology Harvard School of Dental Medicine 188 Longwood Avenue Boston MA 02115 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James R.","family":"Mitchell","sequence":"additional","affiliation":[{"name":"Department of Genetics and Complex Diseases Harvard School of Public Health 655 Huntington Ave Boston MA 02115 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,9,11]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccr.2006.05.027"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1070174"},{"key":"e_1_2_4_4_1","first-page":"227","article-title":"Cockayne's syndrome: literature review and case report","volume":"13","author":"Boraz RA","year":"1991","journal-title":"Pediatr. 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