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标题：Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds
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作者：Markus A. Keller ; Katrin Watschinger ; Karsten Lange 等
期刊名称：JLR Papers In Press
印刷版ISSN：0022-2275
电子版ISSN：1539-7262
出版年度：2012
卷号：53
期号：7
页码：1410-1416
DOI：10.1194/jlr.D025650
语种：English
出版社：American Society for Biochemistry and Molecular Biology
摘要：The lack of fatty aldehyde dehydrogenase function in Sjögren Larsson Syndrome (SLS) patient cells not only impairs the conversion of fatty aldehydes into their corresponding fatty acid but also has an effect on connected pathways. Alteration of the lipid profile in these cells is thought to be responsible for severe symptoms such as ichtyosis, mental retardation, and spasticity. Here we present a novel approach to examine fatty aldehyde metabolism in a time-dependent manner by measuring pyrene-labeled fatty aldehyde, fatty alcohol, fatty acid, and alkylglycerol in the culture medium of living cells using HPLC separation and fluorescence detection. Our results show that in fibroblasts from SLS patients, fatty aldehyde is not accumulating but is converted readily into fatty alcohol. In control cells, in contrast, exclusively the corresponding fatty acid is formed. SLS patient cells did not display a hypersensitivity toward hexadecanal or hexadecanol, but 3-fold lower concentrations of the fatty alcohol than the corresponding fatty aldehyde were needed to induce toxicity in SLS patient and in control cells.
关键词：Sjögren Larsson Syndrome ; fatty aldehyde metabolism ; long chain fatty aldehyde dehydrogenase ; inborn errors of metabolism ; oxidized lipids ; skin ; toxicology