One manner in which eukaryotic cells respond to their environments is by optimizing the composition and proportions of sterols and sphingolipids in membranes. The physical association of the planar ring of sterols with the acyl chains of phospholipids, particularly sphingolipids, produces membrane micro-heterogeneity that is exploited to coordinate several crucial pathways. We hypothesize that these lipid molecules play an integrated role in human disease; when one of the partners is mis-regulated, pathology frequently ensues. Sterols and sphingolipid levels are not coordinated by the action of a single master regulator, however the cross-talk between their metabolic pathways is considerable. We describe our perspectives on the key components of synthesis, catabolism and transport of these lipid partners with an emphasis on evolutionarily conserved reactions that produce disease states when defective.
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http://dx.doi.org/10.1016/j.plipres.2010.03.003 | DOI Listing |
Hepatol Commun
January 2025
Research and Development, Sanofi, Cambridge, Massachusetts, USA.
Background: Acid sphingomyelinase deficiency (ASMD) and Gaucher disease type 1 (GD1) are rare inherited sphingolipid disorders with multisystemic manifestations, including liver disease and dyslipidemia. Despite effective treatments, insufficient disease awareness frequently results in diagnostic delays during which irreversible complications occur. We delineated the shared and distinctive features of hepatic, splenic, and lipoprotein phenotypes in ASMD and GD1.
View Article and Find Full Text PDFMolecules
December 2024
Coimbra Chemistry Center, Institute of Molecular Sciences (CQC-IMS), University of Coimbra, 3004-535 Coimbra, Portugal.
The membrane dipole potential that arises from the interfacial water and constitutive dipolar groups of lipid molecules modulates the interaction of amphiphiles and proteins with membranes. Consequently, its determination for lipid mixtures resembling the existing diversity in biological membranes is very relevant. In this work, the dipole potentials of monolayers, formed at the air-water interface, from pure or mixed lipids (1-palmitoyl-2-oleoyl--glycero-3-phosphocholine (POPC), 1-palmitoyl-2-oleoyl--glycero-3-phosphoethanolamine (POPE), 1-palmitoyl-2-oleoyl--glycero-3-phosphatidyserine (POPS), sphingomyelin (SpM) and cholesterol) were measured and correlated with the mean area per lipid.
View Article and Find Full Text PDFCell Mol Biol Lett
December 2024
Department of Hematology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Add: No.324, Jingwu Road, Jinan, 250021, Shandong, China.
Background: Disorders of lipid metabolism are critical factors in the progression of chronic lymphocytic leukemia (CLL). However, the characteristics of lipid metabolism and related regulatory mechanisms of CLL remain unclear.
Methods: Hence, we identified altered metabolites and aberrant lipid metabolism pathways in patients with CLL by ultra-high-performance liquid chromatography-mass spectrometry-based non-targeted lipidomics.
J Food Sci
December 2024
State Key Laboratory of Food Science and Resources, School of Food Science and Technology, Jiangnan University, Wuxi, PR China.
Recent evidence increasingly indicates that moderate egg consumption (up to one egg per day) poses no significant risk of cardiovascular diseases in healthy individuals, leading researchers to speculate whether other lipids in eggs affect lipid metabolism. In this research, C57BL/6J mice were given chow diets containing egg yolk low-density lipoprotein (LDL) and high-density lipoprotein (HDL), two natural components with different proportions of phospholipids and cholesterol, to explore the effects of the phospholipids-to-cholesterol intake ratio on lipid metabolism. Increases in liver weight and liver index were observed following LDL intake, while body weight and epididymal fat decreased after HDL intake in a dose-dependent manner.
View Article and Find Full Text PDFAm J Physiol Heart Circ Physiol
January 2025
Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, United States.
Farber disease (FD) is an ultrarare, autosomal-recessive, lysosomal storage disorder attributed to gene mutations. FD is characterized by acid ceramidase (ACDase) deficiency and the accumulation of ceramide in various tissues. Classical FD patients typically manifest symptoms including lipogranulomatosis, respiratory complications, and neurological deficits, often leading to mortality during infancy.
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