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- Title
Different Lipid Signature in Fibroblasts of Long-Chain Fatty Acid Oxidation Disorders.
- Authors
Alatibi, Khaled I.; Hagenbuchner, Judith; Wehbe, Zeinab; Karall, Daniela; Ausserlechner, Michael J.; Vockley, Jerry; Spiekerkoetter, Ute; Grünert, Sarah C.; Tucci, Sara; Riboni, Laura
- 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.
- Subjects
FATTY acid oxidation; CARNITINE palmitoyltransferase; LIPIDS; FIBROBLASTS; CERAMIDES
- Publication
Cells (2073-4409), 2021, Vol 10, Issue 5, p1239
- ISSN
2073-4409
- Publication type
Article
- DOI
10.3390/cells10051239