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Different Lipid Signature in Fibroblasts of Long-Chain Fatty Acid Oxidation Disorders.

Authors :
Alatibi KI
Hagenbuchner J
Wehbe Z
Karall D
Ausserlechner MJ
Vockley J
Spiekerkoetter U
GrĂ¼nert SC
Tucci S
Source :
Cells [Cells] 2021 May 18; Vol. 10 (5). Date of Electronic Publication: 2021 May 18.
Publication Year :
2021

Abstract

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.

Details

Language :
English
ISSN :
2073-4409
Volume :
10
Issue :
5
Database :
MEDLINE
Journal :
Cells
Publication Type :
Academic Journal
Accession number :
34069977
Full Text :
https://doi.org/10.3390/cells10051239