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Cuervo, AM (13 July 2011). "Chaperone-mediated autophagy: Dice's 'wild' idea about lysosomal selectivity". Nature Reviews Molecular Cell Biology. 12 (8): 535– 41. doi:10.1038/nrm3150. PMID 21750569. S2CID 23128629. Kaushik, S; Cuervo, AM (2009). "Chapter 19 Methods to Monitor Chaperone-Mediated Autophagy". Autophagy in Mammalian Systems, Part ...
Substrate reduction therapy uses a small molecule to interrupt this multi-step pathway and inhibit the biosynthesis of these compounds. [10] This type of treatment is taken orally. [10] It does not induce an unwanted immune response, and a single type of small molecule could be used to treat many lysosomal storage diseases. [10]
Autophagy degrades damaged organelles, cell membranes and proteins, and insufficient autophagy is thought to be one of the main reasons for the accumulation of damaged cells and aging. [87] Autophagy and autophagy regulators are involved in response to lysosomal damage, often directed by galectins such as galectin-3 and galectin-8.
SNARE proteins – "SNAP REceptors" – are a large protein family consisting of at least 24 members in yeasts and more than 60 members in mammalian and plant cells. [2] [3] [4] The primary role of SNARE proteins is to mediate the fusion of vesicles with the target membrane; this notably mediates exocytosis, but can also mediate the fusion of vesicles with membrane-bound compartments (such as ...
The lysosomal membrane protects the cytosol, and therefore the rest of the cell, from the degradative enzymes within the lysosome. The cell is additionally protected from any lysosomal acid hydrolases that drain into the cytosol, as these enzymes are pH-sensitive and do not function well or at all in the alkaline environment of the cytosol ...
Further, some of the functions of LAMP2 are believed to be protecting the lysosomal membrane from proteolytic enzymes that are within the lysosome itself (as in autodigestion), acting as a receptor into the lysosome for proteins, adhesion (when expressed on the outside surface of the plasma membrane) and signal transduction, both inter- and intra-.
As the disease progresses, it can cause life-threatening liver dysfunction or liver failure. [3] Infants are chronically ill from birth and rarely survive beyond the first year of life. In 2015, an enzyme replacement therapy, sebelipase alfa, was approved in the US and EU. The therapy was additionally approved in Japan in 2016.
Nonetheless, these structures contain endocytic markers even small lysosomal proteins such as cathepsin D. The process is similar in yeast, however the gene names differ. For example, LC3 in mammals is Atg8 in yeast and autophagosomes are generated from Pre-Autophagosomal Structure (PAS) which is distinct from the precursor structures in ...