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...
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Gespeichert in:

Autor*in:	Markus A. Keller [verfasserIn] Katrin Watschinger [verfasserIn] Karsten Lange [verfasserIn] Georg Golderer [verfasserIn] Gabriele Werner-Felmayer [verfasserIn] Albin Hermetter [verfasserIn] Ronald J.A. Wanders [verfasserIn] Ernst R. Werner [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2012

Schlagwörter:	Sjögren Larsson Syndrome fatty aldehyde metabolism long chain fatty aldehyde dehydrogenase inborn errors of metabolism oxidized lipids skin

Übergeordnetes Werk:	In: Journal of Lipid Research - Elsevier, 2021, 53(2012), 7, Seite 1410-1416
Übergeordnetes Werk:	volume:53 ; year:2012 ; number:7 ; pages:1410-1416

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.1194/jlr.D025650

Katalog-ID:	DOAJ015063836

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520			\|a 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.
650		4	\|a Sjögren Larsson Syndrome
650		4	\|a fatty aldehyde metabolism
650		4	\|a long chain fatty aldehyde dehydrogenase
650		4	\|a inborn errors of metabolism
650		4	\|a oxidized lipids
650		4	\|a skin
653		0	\|a Biochemistry
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700	0		\|a Ronald J.A. Wanders \|e verfasserin \|4 aut
700	0		\|a Ernst R. Werner \|e verfasserin \|4 aut
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authorswithroles_txt_mv	Markus A. Keller @@aut@@ Katrin Watschinger @@aut@@ Karsten Lange @@aut@@ Georg Golderer @@aut@@ Gabriele Werner-Felmayer @@aut@@ Albin Hermetter @@aut@@ Ronald J.A. Wanders @@aut@@ Ernst R. Werner @@aut@@
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callnumber-first	Q - Science
author	Markus A. Keller
spellingShingle	Markus A. Keller misc QD415-436 misc Sjögren Larsson Syndrome misc fatty aldehyde metabolism misc long chain fatty aldehyde dehydrogenase misc inborn errors of metabolism misc oxidized lipids misc skin misc Biochemistry Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds
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topic_title	QD415-436 Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds Sjögren Larsson Syndrome fatty aldehyde metabolism long chain fatty aldehyde dehydrogenase inborn errors of metabolism oxidized lipids skin
topic	misc QD415-436 misc Sjögren Larsson Syndrome misc fatty aldehyde metabolism misc long chain fatty aldehyde dehydrogenase misc inborn errors of metabolism misc oxidized lipids misc skin misc Biochemistry
topic_unstemmed	misc QD415-436 misc Sjögren Larsson Syndrome misc fatty aldehyde metabolism misc long chain fatty aldehyde dehydrogenase misc inborn errors of metabolism misc oxidized lipids misc skin misc Biochemistry
topic_browse	misc QD415-436 misc Sjögren Larsson Syndrome misc fatty aldehyde metabolism misc long chain fatty aldehyde dehydrogenase misc inborn errors of metabolism misc oxidized lipids misc skin misc Biochemistry
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title	Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds
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title_full	Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds
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author_browse	Markus A. Keller Katrin Watschinger Karsten Lange Georg Golderer Gabriele Werner-Felmayer Albin Hermetter Ronald J.A. Wanders Ernst R. Werner
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title_sort	studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds
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title_auth	Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds
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.
abstractGer	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.
abstract_unstemmed	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.
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title_short	Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds
url	https://doi.org/10.1194/jlr.D025650 https://doaj.org/article/8fb5c905ce3a4469af76461f11f4d94d http://www.sciencedirect.com/science/article/pii/S0022227520345181 https://doaj.org/toc/0022-2275
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up_date	2024-07-04T01:29:16.306Z
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Studying fatty aldehyde metabolism in living cells with pyrene-labeled compounds

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