Different Lipid Signature in Fibroblasts of Long-Chain Fatty Acid Oxidation Disorders.
Acyl-CoA Dehydrogenase, Long-Chain
/ deficiency
Cardiolipins
/ metabolism
Carnitine O-Palmitoyltransferase
/ deficiency
Case-Control Studies
Cells, Cultured
Ceramides
/ metabolism
Fatty Acids
/ metabolism
Female
Fibroblasts
/ enzymology
Humans
Lipid Metabolism, Inborn Errors
/ enzymology
Lipidomics
Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase
/ deficiency
Male
Metabolism, Inborn Errors
/ enzymology
Metabolome
Oxidation-Reduction
Skin
/ enzymology
Sphingolipids
/ metabolism
Tandem Mass Spectrometry
cardiolipine
ceramides
fatty acid oxidation disorders
lipidomic profiling
sphingomyelins
Journal
Cells
ISSN: 2073-4409
Titre abrégé: Cells
Pays: Switzerland
ID NLM: 101600052
Informations de publication
Date de publication:
18 05 2021
18 05 2021
Historique:
received:
13
04
2021
revised:
15
05
2021
accepted:
17
05
2021
entrez:
2
6
2021
pubmed:
3
6
2021
medline:
27
10
2021
Statut:
epublish
Résumé
Long-chain fatty acid oxidation disorders (lc-FAOD) are a group of diseases affecting the degradation of long-chain fatty acids. In order to investigate the disease specific alterations of the cellular lipidome, we performed undirected lipidomics in fibroblasts from patients with carnitine palmitoyltransferase II, very long-chain acyl-CoA dehydrogenase, and long-chain 3-hydroxyacyl-CoA dehydrogenase. We demonstrate a deep remodeling of mitochondrial cardiolipins. The aberrant phosphatidylcholine/phosphatidylethanolamine ratio and the increased content of plasmalogens and of lysophospholipids support the theory of an inflammatory phenotype in lc-FAOD. Moreover, we describe increased ratios of sphingomyelin/ceramide and sphingomyelin/hexosylceramide in LCHAD deficiency which may contribute to the neuropathic phenotype of LCHADD/mitochondrial trifunctional protein deficiency.
Identifiants
pubmed: 34069977
pii: cells10051239
doi: 10.3390/cells10051239
pmc: PMC8157847
pii:
doi:
Substances chimiques
Cardiolipins
0
Ceramides
0
Fatty Acids
0
Sphingolipids
0
Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase
EC 1.1.1.211
Acyl-CoA Dehydrogenase, Long-Chain
EC 1.3.8.8
Carnitine O-Palmitoyltransferase
EC 2.3.1.21
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : NIDDK NIH HHS
ID : R01 DK078775
Pays : United States
Organisme : Ultragenix
ID : UX007-NCIST217
Organisme : Deutsche Forschungsgemeinschaft
ID : TU492/3-1
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