Osmotic stress is a critical challenge for mammalian cells as loss of water triggered by a hyperosmotic environment promotes harmful protein aggregation and impairs cell survival. How the degradative capacity of cells, in particular the macroautophagy/autophagy-lysosome system, is adapted to meet the ...
In addition to its proteolytic activity, supplementary, γ-secretase independent, functions in the macroautophagy/autophagy-lysosome system have been proposed. Here, we screened for PSENEN-interacting proteins and identified CLN3. Mutations in CLN3 are causative for juvenile neuronal ceroid lipofuscinosis...
Autophagy is a major intracellular degradation system that derives its degradative abilities from the lysosome. The most well-studied form of autophagy is macroautophagy, which delivers cytoplasmic material to lysosomes via the double-membraned autophago
McKeon JE, Sha D, Li L, Chin LS (2015) Parkin-mediated K63-polyubiquitination targets ubiquitin C-terminal hydrolase L1 for degradation by the autophagy-lysosome system. Cell Mol Life Sci 72:1811–1824McKeon JE, Sha D, Li L, Chin L-S (2015) Parkin-mediated K63- polyubiquitination ...
Disruption in the autophagy-lysosome pathway has been documented previously in Fabry disease but its relative contribution to nervous system pathology in Fabry disease is unknown. Using an experimental mouse model of Fabry disease, alpha-galactosidase A deficiency, we examined brain pathology in 20-24...
Potential role of the ubiquitin-proteasome system in atherosclerosis: aspects of a protein quality disease J. Am. Coll. Cardiol. (2008) M. Nowicki et al. No upregulation of lectin-like oxidized low-density lipoprotein receptor-1 in serum-deprived EA.hy926 endothelial cells under oxLDL exposure...
The central nervous system is severely affected. Lysosome is the final degradative organelles for autophagy by which macromolecules and damaged cellular components and organelles are degraded. Impairment in autophagy is a central and common mechanism underlying many LSD...
The Tet-on system is an inducible gene expression system for mammalian cells. In the presence of doxycycline (DOX), the Tet-on PC12 cells overexpress α-syn under the control of a TRE3G promoter29. Tet-on A53T α-syn inducible PC12 cells were treated with 1 µg/mL DOX for 24...
Accumulating evidence suggests that the endolysosomal system is a novel intracellular Ca2+ pool mobilized by the second messenger, nicotinic acid adenine d... GD Dickinson,GC Churchill,E Brailoiu,... 被引量: 29发表: 0年 加载更多 来源期刊 Open Biology 研究点推荐 deregulated autophagy Lysosome-...
Various metabolites can result in damage to podocytes, such as advanced glycation end products (AGEs), which could interact with the receptor of AGE (RAGE) to initiate downstream signaling pathways, such as inflammation, interaction with rennin–angiotensin system (RAS), and oxidative stress, ...