{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T00:24:49Z","timestamp":1790641489759,"version":"4.1.0"},"reference-count":24,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2004,10,7]],"date-time":"2004-10-07T00:00:00Z","timestamp":1097107200000},"content-version":"vor","delay-in-days":8894,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1980,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Because postmortem brain and adrenal tissue from patients with adrenoleukodystrophy (ALD) or adrenomyeloneuropathy (AMN) have been shown to contain abnormally large amounts of very long chain fatty acids (C<jats:sub>24<\/jats:sub> through C<jats:sub>30<\/jats:sub>), we searched for such an abnormality in cultured skin fibroblasts. Total lipid extracts of cultured fibroblasts were hydrolyzed, their fatty acid composition was derermined by gas\u2010liquid chromatography, and the ratio of C<jats:sub>26<\/jats:sub> to C<jats:sub>22<\/jats:sub> fatty acids was calculated.<\/jats:p><jats:p>In 29 control cell lines this ratio was 0.064 \u00b1 0.019. In 5 patients with autopsy\u2010proved ALD the ratio was 0.778 \u00b1 0.139; in 6 patients with clinical features typical of ALD it was 0.764 \u00b1 0.092; in 2 patients with autopsy\u2010proved AMN, 0.890 \u00b1 0.02; and in 2 patients with clinical features typical of AMN, 0.560 \u00b1 0.079. Abnormal ratios were observed in 4 of 5 ALD heterozygotes. In 3 patients in whom the diagnosis of ALD was suspected, an abnormal ratio (0.860) was observed in 1 and normal ratios (0.06 and 0.074) in the 2 others.<\/jats:p>","DOI":"10.1002\/ana.410070607","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T13:14:30Z","timestamp":1104585270000},"page":"542-549","source":"Crossref","is-referenced-by-count":160,"title":["Adrenoleukodystrophy: Elevated C26 fatty acid in cultured skin fibroblasts"],"prefix":"10.1002","volume":"7","author":[{"given":"Hugo W.","family":"Moser","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ann 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