{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,9,17]],"date-time":"2023-09-17T05:10:08Z","timestamp":1694927408301},"reference-count":21,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[1987,11,18]],"date-time":"1987-11-18T00:00:00Z","timestamp":564192000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1988,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Chediak\u2010Higashi syndrome (CHS) cells have been previously observed to exhibit several of the same characteristics as those of xeroderma pigmentosum (XP) and xeroderma pigmentosum variants. Cultured CHS fibroblasts have been examined for altered responses in both depletion of NAD and elevation of diadenosine\u20105\u2032,5\u2034\u2010tetraphosphate (Ap<jats:sub>4<\/jats:sub>A) following DNA damage since both responses have been reported as altered in XP cells and since Ap<jats:sub>4<\/jats:sub>A has been reported as absent from the platelets of CHS patients. Lowering of NAD following UV irradiation occurred in CHS cells in a manner similar to that of control and XP variant cells, but different from that of XP cells. CHS fibroblasts were not found to be deficient in Ap<jats:sub>4<\/jats:sub>A and exhibit basal levels very similar to those of control fibroblasts. No change in Ap<jats:sub>4<\/jats:sub>A pools were observed which correlated with cell growth, in contrast to previously published reports. Furthermore, while Ap<jats:sub>4<\/jats:sub>A levels are not elevated in XP cells, we observe an elevation of Ap<jats:sub>4<\/jats:sub>A pools in CHS cells which mimics the elevations observed in control and XP variant cells. We conclude that:<jats:list>\n<jats:list-item>\n<jats:p>CHS cells more closely resemble control or XP variant cells than XP cells with regard to NAD lowering and Ap<jats:sub>4<\/jats:sub>A elevation following UV irradiation;<\/jats:p><\/jats:list-item>\n<jats:list-item>\n<jats:p>the photosensitivity exhibited by CHS cells is not due to general defects in the synthesis of poly(ADP\u2010ribose) from NAD or in Ap<jats:sub>4<\/jats:sub>A metabolism; and<\/jats:p><\/jats:list-item>\n<jats:list-item>\n<jats:p>alteration of Ap<jats:sub>4<\/jats:sub>A pool size in CHS fibroblasts is inappropriate as a biochemical marker for CHS.<\/jats:p><\/jats:list-item>\n<\/jats:list><\/jats:p>","DOI":"10.1007\/bf01800362","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T17:04:24Z","timestamp":1119373464000},"page":"221-228","source":"Crossref","is-referenced-by-count":4,"title":["Alterations of NAD and adenylyl dinucleotide metabolism in Chediak\u2010Higashi Syndrome fibroblasts"],"prefix":"10.1002","volume":"11","author":[{"given":"J. C.","family":"Baker","sequence":"first","affiliation":[{"name":"Department of Biochemistry University of California  Berkeley CA 94720 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B. N.","family":"Ames","sequence":"additional","affiliation":[{"name":"Department of Biochemistry University of California  Berkeley CA 94720 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1987,11,18]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01800860"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(84)90070-8"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.83.8.2350"},{"key":"e_1_2_1_5_1","unstructured":"Baker J. C.andAmes B. N.Alterations in levels of diadenosine\u20105\u2032 5\u2034\u2010P1 P4\u2010tetraphosphate (Ap4A) following DNA damage in normal human fibroblasts and fibroblasts derived from patients with xeroderma pigmentosum.Mutat. Res.(in press)."},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi00543a006"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-197207000-00001"},{"key":"e_1_2_1_8_1","first-page":"367","article-title":"Inhibition of DNA excision repair in human cells by arabinofuranosyl cytosine: effect on normal and xeroderma pigmentosum cells","volume":"15","author":"Dunn W. C.","year":"1979","journal-title":"Mol. 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