Artikel ; Online: Effects of Triheptanoin on Mitochondrial Respiration and Glycolysis in Cultured Fibroblasts from Neutral Lipid Storage Disease Type M (NLSD-M) Patients.
2023 Band 13, Heft 3
Abstract: Neutral lipid storage disease type M (NLSD-M) is an ultra-rare, autosomal recessive disorder that causes severe skeletal and cardiac muscle damage and lipid accumulation in all body tissues. In this hereditary pathology, the defective action of the ... ...
| Abstract | Neutral lipid storage disease type M (NLSD-M) is an ultra-rare, autosomal recessive disorder that causes severe skeletal and cardiac muscle damage and lipid accumulation in all body tissues. In this hereditary pathology, the defective action of the adipose triglyceride lipase (ATGL) enzyme induces the enlargement of cytoplasmic lipid droplets and reduction in the detachment of mono- (MG) and diglycerides (DG). Although the pathogenesis of muscle fiber necrosis is unknown, some studies have shown alterations in cellular energy production, probably because MG and DG, the substrates of Krebs cycle, are less available. No tests have been tried with medium-chain fatty acid molecules to evaluate the anaplerotic effect in NLSD cells. In this study, we evaluated the in vitro effect of triheptanoin (Dojolvi |
|---|---|
| Mesh-Begriff(e) | Humans ; Lipase/metabolism ; Triglycerides ; Glycolysis ; Fibroblasts/metabolism |
| Chemische Substanzen | triheptanoin (2P6O7CFW5K) ; Lipase (EC 3.1.1.3) ; Triglycerides |
| Sprache | Englisch |
| Erscheinungsdatum | 2023-03-01 |
| Erscheinungsland | Switzerland |
| Dokumenttyp | Journal Article ; Research Support, Non-U.S. Gov't |
| ZDB-ID | 2701262-1 |
| ISSN | 2218-273X ; 2218-273X |
| ISSN (online) | 2218-273X |
| ISSN | 2218-273X |
| DOI | 10.3390/biom13030452 |
| Datenquelle | MEDical Literature Analysis and Retrieval System OnLINE |
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