Different Lipid Signature in Fibroblasts of Long-Chain Fatty Acid Oxidation Disorders
| Autor: | Alatibi, Khaled I., Hagenbuchner, Judith, Wehbe, Zeinab, Karall, Daniela, Ausserlechner, Michael J., Vockley, Jerry, Spiekerkoetter, Ute, Grünert, Sarah C., Tucci, Sara |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Male
Cardiolipins QH301-705.5 fatty acid oxidation disorders Article Lipid Metabolism Inborn Errors Tandem Mass Spectrometry Humans Biology (General) Cells Cultured Skin Sphingolipids ceramides Carnitine O-Palmitoyltransferase Acyl-CoA Dehydrogenase Long-Chain Fatty Acids Fibroblasts sphingomyelins Case-Control Studies Lipidomics Metabolome lipidomic profiling Female Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase lipids (amino acids peptides and proteins) Oxidation-Reduction Metabolism Inborn Errors cardiolipine |
| Zdroj: | Cells Volume 10 Issue 5 Cells, Vol 10, Iss 1239, p 1239 (2021) |
| ISSN: | 2073-4409 |
| DOI: | 10.3390/cells10051239 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|