PMID: 2117919Aug 1, 1990Paper

Metabolism of saturated and polyunsaturated very-long-chain fatty acids in fibroblasts from patients with defects in peroxisomal beta-oxidation

The Biochemical Journal
J M StreetA Poulos

Abstract

The metabolism of [1-14C]lignoceric acid (C24:0) and [1-14C]tetracosatetraenoic acid (C24:4, n-6) was studied in normal skin fibroblast cultures and in cultures from patients with defects in peroxisomal beta-oxidation (but normal peroxisomal numbers). Cells from X-linked adrenoleukodystrophy (ALD) patients with a presumed defect in a peroxisomal acyl-CoA synthetase, specific for fatty acids of carbon chain lengths greater than 22 (very-long-chain fatty acids; VLCFA), showed a relatively normal production of radiolabelled CO2 and water-soluble metabolites from [1-14C]C24:0. However, the products of synthesis from acetate de novo (released by beta-oxidation), i.e. C16 and C18 fatty acids, were decreased, and carbon chain elongation of the fatty acid was increased. In contrast, cell lines from two patients with an unidentified lesion in peroxisomal beta-oxidation (peroxisomal disease, PD) showed a marked deficiency in CO2 and water-soluble metabolite production, a decreased synthesis of C16 and C18 fatty acids and an increase in carbon chain elongation. The relatively normal beta-oxidation activity of ALD cells appears to be related to low uptake of substrate, as a defect in beta-oxidation is apparent when measurements are perform...Continue Reading

Citations

Jan 13, 2009·Clinica Chimica Acta; International Journal of Clinical Chemistry·Aya OkahashiTakashi Shimizu
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
Jun 16, 2012·Molecular BioSystems·Swagatika SahooInes Thiele
Apr 23, 1993·Biochimica Et Biophysica Acta·M Guzmán, M J Geelen
Sep 11, 1991·Biochimica Et Biophysica Acta·H OsmundsenJ I Pedersen

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Adrenoleukodystrophy (ALD), the most frequent peroxisomal disorder, is an X-linked disorder caused by a defect in the metabolism of long chain fatty acids leading to demyelination, neurodegeneration, and death. Here is the latest research.