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Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds
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 respo...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The American Society for Biochemistry and Molecular
Biology
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3371253/ https://www.ncbi.nlm.nih.gov/pubmed/22508945 http://dx.doi.org/10.1194/jlr.D025650 |
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author | Keller, Markus A. Watschinger, Katrin Lange, Karsten Golderer, Georg Werner-Felmayer, Gabriele Hermetter, Albin Wanders, Ronald J. A. Werner, Ernst R. |
author_facet | Keller, Markus A. Watschinger, Katrin Lange, Karsten Golderer, Georg Werner-Felmayer, Gabriele Hermetter, Albin Wanders, Ronald J. A. Werner, Ernst R. |
author_sort | Keller, Markus A. |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-3371253 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | The American Society for Biochemistry and Molecular
Biology |
record_format | MEDLINE/PubMed |
spelling | pubmed-33712532013-07-01 Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds Keller, Markus A. Watschinger, Katrin Lange, Karsten Golderer, Georg Werner-Felmayer, Gabriele Hermetter, Albin Wanders, Ronald J. A. Werner, Ernst R. J Lipid Res Methods 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. The American Society for Biochemistry and Molecular Biology 2012-07 /pmc/articles/PMC3371253/ /pubmed/22508945 http://dx.doi.org/10.1194/jlr.D025650 Text en Copyright © 2012 by the American Society for Biochemistry and Molecular Biology, Inc. http://creativecommons.org/licenses/by-nc/3.0/ Author's Choice—Final version full access. Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) applies to Author Choice Articles |
spellingShingle | Methods Keller, Markus A. Watschinger, Katrin Lange, Karsten Golderer, Georg Werner-Felmayer, Gabriele Hermetter, Albin Wanders, Ronald J. A. Werner, Ernst R. Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds |
title | Studying fatty aldehyde metabolism in living cells with pyrene-labeled
compounds |
title_full | Studying fatty aldehyde metabolism in living cells with pyrene-labeled
compounds |
title_fullStr | Studying fatty aldehyde metabolism in living cells with pyrene-labeled
compounds |
title_full_unstemmed | Studying fatty aldehyde metabolism in living cells with pyrene-labeled
compounds |
title_short | Studying fatty aldehyde metabolism in living cells with pyrene-labeled
compounds |
title_sort | studying fatty aldehyde metabolism in living cells with pyrene-labeled
compounds |
topic | Methods |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3371253/ https://www.ncbi.nlm.nih.gov/pubmed/22508945 http://dx.doi.org/10.1194/jlr.D025650 |
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