{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T17:35:09Z","timestamp":1760981709956,"version":"build-2065373602"},"reference-count":25,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[1989,4,1]],"date-time":"1989-04-01T00:00:00Z","timestamp":607392000000},"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":[[1989,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The fatty acid composition of red blood cells, fibroblasts, forebrain, liver and kidney were studied in a 3\u2010month\u2010old infant who died from Zellweger Syndrome, and the results were compared with those of age\u2010matched controls. Besides a typical increase in the very long chain fatty acids 26\u22360 and 26\u22361 and a great reduction in the plasmalogen levels, confirming the diagnosis of Zellweger Syndrome, some striking changes in the polyunsaturated fatty acid patterns were discovered. The most important was a very drastic decrease in the values of 22\u22366\u03c93 and 22\u22365\u03c96, the two products of \u03944\u2010desaturation. In the kidney, the level of 22\u22366\u03c93 fell below that of 26\u22360. Consequently, the ratio 26\u22360\/22\u22366\u03c93 (and 26\u22361\/22\u22366\u03c93) was most useful in emphasizing the fatty acid anomalies, especially in renal tissue, where the 26\u22360\/22\u22366\u03c93 ratio increased to almost 200 times the normal values. Other significant, although less consistent fatty acid alterations were increases in 18\u22362\u03c96, 18\u22363\u03c96, 20\u22363\u03c96, 18\u22364\u03c93 and 20\u22364\u03c93, and a decrease in 20\u22364\u03c96 in some tissues. The existence is proposed of a new enzyme defect in peroxisomal disorders, involving the desaturase system of long chain polyunsaturated fatty acids.<\/jats:p>","DOI":"10.1007\/bf02535160","type":"journal-article","created":{"date-parts":[[2006,11,29]],"date-time":"2006-11-29T03:04:04Z","timestamp":1164769444000},"page":"261-265","source":"Crossref","is-referenced-by-count":56,"title":["Polyunsaturated fatty acid changes suggesting a new enzymatic defect in Zellweger Syndrome"],"prefix":"10.1002","volume":"24","author":[{"given":"Manuela","family":"Martinez","sequence":"first","affiliation":[{"name":"Autonomous University of Barcelona, School of Medicine Hospital Infantil Vall d'Hebron Gran Via 750, 6\u20101 Barcelona 08013 Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1989,4]]},"reference":[{"key":"e_1_2_2_2_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.182.4107.62"},{"key":"e_1_2_2_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)86607-3"},{"key":"e_1_2_2_4_1","first-page":"344","volume":"151","author":"Brown F.R.","year":"1982","journal-title":"Johns Hopkins Med. 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