{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T22:51:09Z","timestamp":1791413469386,"version":"4.3.3"},"reference-count":39,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2015,1,8]],"date-time":"2015-01-08T00:00:00Z","timestamp":1420675200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Photochem &amp; Photobiology"],"published-print":{"date-parts":[[2015,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    In 1968, Dr. James Cleaver reported defective\n                    <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content>\n                    repair in cultured cells from patients with xeroderma pigmentosum. This link between clinical disease and molecular pathophysiology has sparked interest in understanding not only the clinical characteristics of sun sensitivity, damage and cancer that occurred in\n                    <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>\n                    patients but also the mechanisms underlying the damage and repair. While affected patients are rare, their exaggerated\n                    <jats:styled-content style=\"fixed-case\">UV<\/jats:styled-content>\n                    damage provides a window into the workings of\n                    <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content>\n                    repair. These studies have clarified the importance of a functioning\n                    <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content>\n                    repair system to the maintenance of skin and neurologic health in the general population. Understanding the role of damage in causing cancer, neurologic degeneration, hearing loss and internal cancers provides an opportunity for prevention and treatment. Characterizing complementation groups pointed to the importance of different underlying genes. Studying differences in cancer age of onset and underlying molecular signatures in cancers occurring either in\n                    <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>\n                    patients or the general population has led to insights into differences in carcinogenic mechanisms. The accelerated development of cancers in\n                    <jats:styled-content style=\"fixed-case\">XP<\/jats:styled-content>\n                    has been used as a model to discover new cancer chemopreventive agents. An astute insight can be a \u201ctipping point\u201d triggering decades of productive inquiry.\n                  <\/jats:p>","DOI":"10.1111\/php.12345","type":"journal-article","created":{"date-parts":[[2014,9,15]],"date-time":"2014-09-15T04:33:48Z","timestamp":1410755628000},"page":"452-459","source":"Crossref","is-referenced-by-count":49,"title":["Forty Years of Research on Xeroderma Pigmentosum at the\n                    <scp>US<\/scp>\n                    National Institutes of Health"],"prefix":"10.1111","volume":"91","author":[{"given":"Kenneth H.","family":"Kraemer","sequence":"first","affiliation":[{"name":"Dermatology Branch Center for Cancer Research National Cancer Institute National Institutes of Health Bethesda MD"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John J.","family":"DiGiovanna","sequence":"additional","affiliation":[{"name":"Dermatology Branch Center for Cancer Research National Cancer Institute National Institutes of Health Bethesda MD"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,1,8]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/218652a0"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2836(64)80216-3"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.168.3938.1477"},{"key":"e_1_2_13_5_1","doi-asserted-by":"crossref","first-page":"2073","DOI":"10.1001\/jama.1969.03150240093011","article-title":"Xeroderma pigmentosum. 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