{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T15:09:05Z","timestamp":1786979345790,"version":"build-2736575974"},"reference-count":19,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[1993,3,1]],"date-time":"1993-03-01T00:00:00Z","timestamp":730944000000},"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":[[1993,3]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    The oxidation of pristanic and phytanic acids by human skin fibroblasts was compared to that of their synthetic analogues, 2\u2010methylpalmitic and 3\u2010methylmargaric acids. The synthetic compounds and natural substrates were degraded at comparable rates in control and X\u2010linked adrenoleukodystrophy fibroblasts. The \u03b1\u2010decarboxylation of 3\u2010methylmargaric acid, similarly to that of phytanic acid, was affected in Refsum disease and Zellweger syndrome, but not in X\u2010linked adrenoleukodystrophy. The \u03b2\u2010oxidation of 2\u2010methylpalmitic acid, similarly to that of pristanic acid, was deficient in fibroblasts derived from patients suffering from Zellweger syndrome, confirming the importance of peroxisomes in the breakdown of 2\u2010methyl\u2010branched fatty acids. No deficiency was observed in fibroblasts from X\u2010linked adrenoleukodystrophy patients. The 1\u2010\n                    <jats:sup>14<\/jats:sup>\n                    C\u2010labelled 2\u2010 and 3\u2010methyl\u2010branched fatty acids, which are easier to synthesize that the natural analogues, are therefore valuable tools for the diagnosis of human peroxisomal disorders.\n                  <\/jats:p>","DOI":"10.1007\/bf00710285","type":"journal-article","created":{"date-parts":[[2004,12,1]],"date-time":"2004-12-01T07:15:16Z","timestamp":1101885316000},"page":"381-391","source":"Crossref","is-referenced-by-count":39,"title":["The deficient degradation of synthetic 2\u2010 and 3\u2010methyl\u2010branched fatty acids in fibroblasts from patients with peroxisomal disorders"],"prefix":"10.1002","volume":"16","author":[{"given":"P. P.","family":"Van Veldhoven","sequence":"first","affiliation":[{"name":"Afdeling Farmakologie, Herestraat Katolieke Universiteit Leuven \u2014 Campus Gasthuisberg Leuven B\u20103000 Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Huang","sequence":"additional","affiliation":[{"name":"Afdeling Farmakologie, Herestraat Katolieke Universiteit Leuven \u2014 Campus Gasthuisberg Leuven B\u20103000 Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. J.","family":"Eyssen","sequence":"additional","affiliation":[{"name":"Rega Instituut \u2010 Minderbroederstraat Leuven B\u20103000 Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. 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