Method for Measurement of Peroxisomal Very Long-Chain Fatty Acid Beta-Oxidation and De Novo C26:0 Synthesis Activity in Living Cells Using Stable-Isotope Labeled Docosanoic Acid.

Methods Mol Biol

Laboratory Genetic Metabolic Diseases (F0-226), Departments of Pediatrics and Clinical Chemistry, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.

Published: May 2017

Peroxisomes are present in virtually every eukaryotic cell type with the exception of the mature erythrocyte. In higher eukaryotes, one of the main functions of peroxisomes is lipid metabolism by means of beta-oxidation of very long-chain fatty acids (VLCFA; ≥22 carbon atoms). A dysfunction in peroxisomal VLCFA beta-oxidation results in elevated VLCFA levels in cells, tissue, and plasma. Here, we describe a straightforward and sensitive method to measure peroxisomal beta-oxidation capacity in living cells using stable-isotope labeled docosanoic acid (D-C22:0).

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http://dx.doi.org/10.1007/978-1-4939-6937-1_5DOI Listing

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