{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T00:45:52Z","timestamp":1761007552678,"version":"build-2065373602"},"reference-count":37,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2000,10,1]],"date-time":"2000-10-01T00:00:00Z","timestamp":970358400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Lipids"],"published-print":{"date-parts":[[2000,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Cerebronic acid (2\u2010hydroxytetracosanoic acid), an \u03b1\u2010hydroxy very long\u2010chain fatty acid (VLCFA) and a component of cerebrosides and sulfatides, is unique to nervous tissues. Studies were carried out to identify the pathway and the subcellular site involved in the oxidation of cerebronic acid. The results from these studies revealed that cerebronic acid was catabolized by \u03b1\u2010oxidation to CO<jats:sub>2<\/jats:sub> and tricosanoic acid (23:0). Studies with subcellular fractions indicated that cerebronic acid was \u03b1\u2010oxidized in fractions having particulate bound catalase and enzyme systems for the \u03b2\u2010oxidation of VLCFA (e.g., lignoceric acid), suggesting peroxisomes as the subcellular organelle responsible for \u03b1\u2010oxidation of cerebronic acid. Etomoxir, an inhibitor of mitochondrial fatty acid oxidation, had no effect on cerebronic acid \u03b1\u2010oxidation. Further, cerebronic acid oxidation was found to be dependent on the presence of NAD<jats:sup>+<\/jats:sup> but not FAD, NADPH, ATP, Mg<jats:sup>2+<\/jats:sup>, or CoASH. Intraorganellar localization studies indicated that the enzyme system for the \u03b1\u2010oxidation of cerebronic acid was associated with the peroxisomal limiting membranes. Studies on cultured fibroblasts from normal subjects and patients with peroxisomal disorders indicated an impairment of \u03b1\u2010oxidation of cerebronic acid in cell lines that lack peroxisomes [e.g., Zellweger syndrome (ZS)]. On the other hand, \u03b1\u2010oxidation of cerebronic acid was found to be normal in cell lines from X\u2010linked adrenoleukodystrophy, adult Refsum disease, and rhizomelic chondrodysplasia punctata. Our results clearly demonstrate that \u03b1\u2010oxidation of \u03b1\u2010hydroxy VLCFA (cerebronic acid) is a peroxisomal function and that this oxidation is impaired in ZS. Furthermore, this \u03b1\u2010oxidation enzyme system is distinct from the one for the \u03b1\u2010oxidation of \u03b2\u2010carbon branched\u2010chain fatty acids (e.g., phytanic acid).<\/jats:p>","DOI":"10.1007\/s11745-000-0628-5","type":"journal-article","created":{"date-parts":[[2006,11,13]],"date-time":"2006-11-13T12:34:36Z","timestamp":1163421276000},"page":"1127-1133","source":"Crossref","is-referenced-by-count":11,"title":["Identification of the pathway of \u03b1\u2010oxidation of cerebronic acid in peroxisomes"],"prefix":"10.1002","volume":"35","author":[{"given":"Rajat","family":"Sandhir","sequence":"first","affiliation":[{"name":"Department of Pediatrics Medical University of South Carolina 171 Ashley Ave. Charleston SC 29425"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mushfiquddin","family":"Khan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Medical University of South Carolina 171 Ashley Ave. Charleston SC 29425"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Inderjit","family":"Singh","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Medical University of South Carolina 171 Ashley Ave. Charleston SC 29425"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2000,10]]},"reference":[{"key":"e_1_2_2_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)40339-6"},{"key":"e_1_2_2_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00223361"},{"key":"e_1_2_2_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(82)90296-7"},{"key":"e_1_2_2_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0163-7827(95)00011-9"},{"key":"e_1_2_2_6_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1006883229684"},{"key":"e_1_2_2_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)82160-3"},{"key":"e_1_2_2_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)64013-X"},{"key":"e_1_2_2_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-3002(63)90726-1"},{"key":"e_1_2_2_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)51748-8"},{"key":"e_1_2_2_11_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.81.13.4203"},{"key":"e_1_2_2_12_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI113271"},{"key":"e_1_2_2_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1995.545zz.x"},{"key":"e_1_2_2_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)32471-8"},{"key":"e_1_2_2_15_1","doi-asserted-by":"publisher","DOI":"10.1021\/jo01118a026"},{"key":"e_1_2_2_16_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.85.20.7647"},{"key":"e_1_2_2_17_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.85.3.890"},{"key":"e_1_2_2_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00234165"},{"key":"e_1_2_2_19_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj0920179"},{"key":"e_1_2_2_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)44883-1"},{"key":"e_1_2_2_21_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.61.1.188"},{"key":"e_1_2_2_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-9861(85)90642-3"},{"key":"e_1_2_2_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/0925-4439(92)90072-U"},{"key":"e_1_2_2_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)37973-6"},{"key":"e_1_2_2_25_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.93.1.103"},{"key":"e_1_2_2_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(17)34166-2"},{"key":"e_1_2_2_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)81113-9"},{"key":"e_1_2_2_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2760(68)90113-6"},{"key":"e_1_2_2_29_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005476631419"},{"key":"e_1_2_2_30_1","doi-asserted-by":"publisher","DOI":"10.1042\/bst0280141"},{"key":"e_1_2_2_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)42022-X"},{"key":"e_1_2_2_32_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1097-185"},{"key":"e_1_2_2_33_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1097-190"},{"key":"e_1_2_2_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(98)00575-4"},{"key":"e_1_2_2_35_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0497-369"},{"key":"e_1_2_2_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)41399-9"},{"key":"e_1_2_2_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/0925-4439(94)90092-2"},{"key":"e_1_2_2_38_1","doi-asserted-by":"publisher","DOI":"10.1177\/095632029600700303"}],"container-title":["Lipids"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/aocs.onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s11745-000-0628-5","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T14:03:29Z","timestamp":1760969009000},"score":1,"resource":{"primary":{"URL":"https:\/\/aocs.onlinelibrary.wiley.com\/doi\/10.1007\/s11745-000-0628-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2000,10]]},"references-count":37,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2000,10]]}},"alternative-id":["10.1007\/s11745-000-0628-5"],"URL":"https:\/\/doi.org\/10.1007\/s11745-000-0628-5","archive":["Portico"],"relation":{},"ISSN":["0024-4201","1558-9307"],"issn-type":[{"type":"print","value":"0024-4201"},{"type":"electronic","value":"1558-9307"}],"subject":[],"published":{"date-parts":[[2000,10]]}}}