{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T00:09:29Z","timestamp":1779149369980,"version":"3.51.4"},"reference-count":0,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2008,1,18]],"date-time":"2008-01-18T00:00:00Z","timestamp":1200614400000},"content-version":"vor","delay-in-days":2970,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IUBMB Life"],"published-print":{"date-parts":[[1999,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Alkyl\u2010dihydroxyacetonephosphate synthase (alkyl\u2010DHAP synthase) is a peroxisomal enzyme that plays a key role in ether phospholipid biosynthesis. To determine the turnover of alkyl\u2010DHAP synthase in several peroxisomal disorders, pulse\u2010chase experiments were performed. In control fibroblasts, mature alkyl\u2010DHAP synthase displayed a half\u2010life of 23 +\/\u2010 12 h. In Zellweger syndrome and rhizomelic chondrodysplasia punctata fibroblast cell lines, in which alkyl\u2010DHAP synthase cannot be imported into peroxisomes, the enzyme was mainly detected in its precursor form. This precursor form showed a much shorter half\u2010life, 5 +\/\u2010 2 h. In contrast, when the precursor protein accumulated inside the peroxisome of a particular neonatal adrenoleukodystrophy cell line in which processing does not take place, a half\u2010life of 18 +\/\u2010 8 h, resembling that of the mature protein in controls, was observed. In a cell line from a patient with a single deficiency in the activity of alkyl\u2010DHAP synthase, the mature form was detected and its radioactivity decreased with a half\u2010life of 16 +\/\u2010 7 h. Collectively, these results provide an explanation for the instability of alkyl\u2010DHAP synthase outside its target organelle. Additionally, they indicate that both the precursor and mature form of alkyl\u2010DHAP synthase exhibit considerable intraperoxisomal turnover.<\/jats:p>","DOI":"10.1080\/713803571","type":"journal-article","created":{"date-parts":[[2007,7,7]],"date-time":"2007-07-07T11:49:33Z","timestamp":1183808973000},"page":"635-639","source":"Crossref","is-referenced-by-count":4,"title":["Stability of Alkyl\u2010Dihydroxyacetonephosphate Synthase in Human Control and Peroxisomal Disorder Fibroblasts"],"prefix":"10.1002","volume":"48","author":[{"given":"Jan","family":"Biermann","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeannette","family":"Gootjes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronald J. A.","family":"Wanders","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Henk Van Den","family":"Bosch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2008,1,18]]},"container-title":["IUBMB Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1080%2F713803571","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/iubmb.onlinelibrary.wiley.com\/doi\/pdf\/10.1080\/713803571","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,29]],"date-time":"2023-10-29T00:18:08Z","timestamp":1698538688000},"score":1,"resource":{"primary":{"URL":"https:\/\/iubmb.onlinelibrary.wiley.com\/doi\/10.1080\/713803571"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1999,12]]},"references-count":0,"journal-issue":{"issue":"6","published-print":{"date-parts":[[1999,12]]}},"alternative-id":["10.1080\/713803571"],"URL":"https:\/\/doi.org\/10.1080\/713803571","archive":["Portico"],"relation":{},"ISSN":["1521-6543","1521-6551"],"issn-type":[{"value":"1521-6543","type":"print"},{"value":"1521-6551","type":"electronic"}],"subject":[],"published":{"date-parts":[[1999,12]]}}}