Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds |
| |
Authors: | Keller Markus A Watschinger Katrin Lange Karsten Golderer Georg Werner-Felmayer Gabriele Hermetter Albin Wanders Ronald J A Werner Ernst R |
| |
Institution: | Division of Biological Chemistry, Biocenter, Innsbruck Medical University, 6020 Innsbruck, Austria. |
| |
Abstract: | 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. |
| |
Keywords: | Sjögren Larsson Syndrome fatty aldehyde metabolism long chain fatty aldehyde dehydrogenase inborn errors of metabolism oxidized lipids skin toxicology |
本文献已被 ScienceDirect PubMed 等数据库收录! |
|