Lysosomes are cytoplasmic organelles delimited by a single membrane and filled with a variety of hydrolytic enzymes active at acidic pH and collectively capable to degrade the vast majority of macromolecules entering lysosomes via endocytosis, phagocytosis or autophagy. In this review, we describe the lipid composition and the dynamic properties of lysosomal membrane, the main delivery pathways of lipids to lysosomes and their catabolism inside lysosomes. Then, we present the consequences of a lipid accumulation as seen in various lysosomal storage diseases on lysosomal functions. Finally, we discuss about the possible involvement of lysosomes in lipotoxicity.
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http://dx.doi.org/10.2174/138920012803762684 | DOI Listing |
Sci Rep
December 2024
College of Animal Science and Technology, Shandong Agricultural University, Tai'an, 271018, China.
VPS28 (vacuolar protein sorting 28) is a subunit of the endosomal sorting complexes required for transport (ESCRTs) and is involved in ubiquitination. Ubiquitination is a critical system for protein degradation in eukaryotes. Considering the recent findings on the role of ubiquitination in the regulation of lipid metabolism, we hypothesized that VPS28 might affect the expression of genes involved in milk fat synthesis.
View Article and Find Full Text PDFOrphanet J Rare Dis
December 2024
Department of Internal Medicine D, and Interdisciplinary Fabry Center (IFAZ), University Hospital Muenster, Muenster, Germany.
Background: The aim of our multicenter study was to investigate the implementation of the European Fabry guidelines on therapeutic recommendations in female patients with Fabry disease (FD) and to analyze the impact of enzyme replacement therapy (ERT) in treated and untreated females.
Results: Data from 3 consecutive visits of 159 female FD patients from 6 Fabry centers were retrospectively analyzed. According to their treatment, patients were separated in 3 groups (untreated, n = 71; newly ERT-treated, n = 47; long-term ERT-treated, n = 41).
Sci Rep
December 2024
Department of Chemistry and Biochemistry, Charles E. Schmidt College of Science, Florida Atlantic University, 777 Glades Rd, Boca Raton, FL, 33431, USA.
We present novel fluorescent cholesteryl probes (CNDs) with a modular design based on the solvatochromic 1,8-phthalimide scaffold. We have explored how different modules-linkers and head groups-affect the ability of these probes to integrate into lipid membranes and how they distribute intracellularly in mouse astrocytes and fibroblasts targeting lysosomes and lipid droplets. Each compound was assessed for its solvatochromic behavior in organic solvents and model membranes.
View Article and Find Full Text PDFOsteoarthritis Cartilage
December 2024
Department of Oral Anatomy and Physiology and TMD, College of Stomatology, the Fourth Military Medical University. Xi'an, China; Shanghai Key Laboratory of Craniomaxillofacial Development and Diseases, Shanghai Stomatological Hospital, Fudan University, Shanghai, China. Electronic address:
Objective: Abnormal mechanical stress is intimately coupled with osteoarthritis (OA). Microtubules play a vital role in the regulation of mechanotransduction and intracellular transport. The purpose of the present study was to investigate the impact of stress-induced microtubule impairment on intracellular transport and lipid droplet (LD) accumulation in chondrocytes.
View Article and Find Full Text PDFMar Drugs
December 2024
G.B. Elyakov Pacific Institute of Bioorganic Chemistry, Far-Eastern Branch of the Russian Academy of Sciences, 690022 Vladivostok, Russia.
Liposomal drug delivery systems are successfully used in various fields of medicine for external and systemic applications. Marine organisms contain biologically active substances that have a unique structure and exhibit a wide range of biological activities. Polysaccharide of red seaweed (carrageenan (CRG)), and water-insoluble sea urchin pigment (echinochrome (Ech)) interact with each other and form a stable complex.
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