Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Keller, Markus A., Watschinger, Katrin, Lange, Karsten, Golderer, Georg, Werner-Felmayer, Gabriele, Hermetter, Albin, Wanders, Ronald J. A., Werner, Ernst R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The American Society for Biochemistry and Molecular Biology 2012
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
_version_ 1782235182852472832
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
work_keys_str_mv AT kellermarkusa studyingfattyaldehydemetabolisminlivingcellswithpyrenelabeledcompounds
AT watschingerkatrin studyingfattyaldehydemetabolisminlivingcellswithpyrenelabeledcompounds
AT langekarsten studyingfattyaldehydemetabolisminlivingcellswithpyrenelabeledcompounds
AT golderergeorg studyingfattyaldehydemetabolisminlivingcellswithpyrenelabeledcompounds
AT wernerfelmayergabriele studyingfattyaldehydemetabolisminlivingcellswithpyrenelabeledcompounds
AT hermetteralbin studyingfattyaldehydemetabolisminlivingcellswithpyrenelabeledcompounds
AT wandersronaldja studyingfattyaldehydemetabolisminlivingcellswithpyrenelabeledcompounds
AT wernerernstr studyingfattyaldehydemetabolisminlivingcellswithpyrenelabeledcompounds