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Oxidation of these fatty acids is deficient in a number of different peroxisomal disorders, including the disorders of peroxisome biogenesis (Zellweger syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease), X\u2010linked adrenoleukodystrophy and a number of other disorders of peroxisomal \u03b2\u2010oxidation of known and unknown aetiology. Accurate measurement of peroxisomal fatty acid oxidation is of utmost importance for correct postnatal and prenatal diagnosis of these disorders. In this paper we describe a straightforward and accurate assay method to measure the \u03b2\u2010oxidation of palmitic acid (C<jats:sub>16:0<\/jats:sub>), hexacosanoic acid (C<jats:sub>26:0<\/jats:sub>) and pristanic acid in intact fibroblasts.<\/jats:p>","DOI":"10.1007\/bf00711434","type":"journal-article","created":{"date-parts":[[2004,11,30]],"date-time":"2004-11-30T10:35:54Z","timestamp":1101810954000},"page":"113-124","source":"Crossref","is-referenced-by-count":84,"title":["Measurement of peroxisomal fatty acid \u03b2\u2010oxidation in cultured human skin fibroblasts"],"prefix":"10.1002","volume":"18","author":[{"given":"R. J. A.","family":"Wanders","sequence":"first","affiliation":[{"name":"Departments of Pediatrics and Clinical Chemistry, Laboratory of Pediatric Clinical Biochemistry, Section for Clinical Enzymology University Hospital Amsterdam  PO Box 22700 Meibergdreef 9 Amsterdam 1100 DE The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Denis","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Clinical Chemistry, Laboratory of Pediatric Clinical Biochemistry, Section for Clinical Enzymology University Hospital Amsterdam  PO Box 22700 Meibergdreef 9 Amsterdam 1100 DE The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. P. N.","family":"Ruiter","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Clinical Chemistry, Laboratory of Pediatric Clinical Biochemistry, Section for Clinical Enzymology University Hospital Amsterdam  PO Box 22700 Meibergdreef 9 Amsterdam 1100 DE The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. B. H.","family":"Schutgens","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Clinical Chemistry, Laboratory of Pediatric Clinical Biochemistry, Section for Clinical Enzymology University Hospital Amsterdam  PO Box 22700 Meibergdreef 9 Amsterdam 1100 DE The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C. W. T.","family":"van Roermund","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Clinical Chemistry, Laboratory of Pediatric Clinical Biochemistry, Section for Clinical Enzymology University Hospital Amsterdam  PO Box 22700 Meibergdreef 9 Amsterdam 1100 DE The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B. S.","family":"Jacobs","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Clinical Chemistry, Laboratory of Pediatric Clinical Biochemistry, Section for Clinical Enzymology University Hospital Amsterdam  PO Box 22700 Meibergdreef 9 Amsterdam 1100 DE The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1995,1]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01959531"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01804226"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0925-4439(94)90057-4"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)38412-1"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI115401"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)37697-5"},{"key":"e_1_2_1_8_1","first-page":"339","article-title":"Peroxisomal 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