PMID: 2504148Jun 15, 1989Paper

Metabolism of saturated and polyunsaturated fatty acids by normal and Zellweger syndrome skin fibroblasts

The Biochemical Journal
J M StreetA Poulos

Abstract

The metabolism of 1-11C-labelled derivatives of palmitic (C16:0), arachidonic (C20:4,n-6) lignoceric (C21:0) and tetracosatetraenoic (C24:4,n-6) acids was studied in normal skin fibroblast cultures and in cultures of fibroblasts from peroxisome-deficient (Zellweger's syndrome) patients. Radiolabelled products of the fatty acids included carbon dioxide. C14-24 saturated and mono-unsaturated fatty acids formed from released acetate either by synthesis de novo or by elongation of endogenous fatty acids, fatty acids formed by 2-6-carbon elongation of added substrates, and a number of water-soluble compounds, some of which were tentatively identified as the amino acids glutamine, glutamic acid and asparagine. The labelled amino acids were found predominantly in the culture medium. Zellweger's syndrome fibroblasts showed a marked decrease in radiolabelled carbon dioxide and water-soluble-product formation from (I-14C)-labelled arachidonic, tetracosatetraenoic and lignoceric acids but not from [I-14C]palmitic acid, and the production of radiolabelled C14-18 fatty acids was also diminished. However, the elongation of individual fatty acids was either normal or above normal. Our data support the view that the oxidation of 20:4, 24:4 and...Continue Reading

Citations

Aug 30, 2008·Proceedings of the National Academy of Sciences of the United States of America·Martin-Paul AgbagaRobert E Anderson
Mar 20, 2010·Journal of Lipid Research·Martin-Paul AgbagaRobert E Anderson
Jul 1, 1991·TAG. Theoretical and applied genetics. Theoretische und angewandte Genetik·D W James, H K Dooner
Nov 21, 2017·Translational Science of Rare Diseases·Catherine ArgyriouNancy Braverman
Nov 25, 1998·Molecular Aspects of Medicine·R J Wanders, J M Tager

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