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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.
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 ...
Probable cation-transporting ATPase 13A2 is an enzyme that in humans is encoded by the ATP13A2 gene that is involved in the transport of divalent transition metal cations. [5] [6] [7] It appears to protect cells from manganese [8] and zinc toxicity, [9] possibly by causing cellular efflux and/or lysosomal sequestration; and from iron toxicity, possibly by preserving lysosome integrity against ...
65114 Ensembl ENSG00000069329 ENSMUSG00000031696 UniProt Q96QK1 Q9EQH3 RefSeq (mRNA) NM_018206 NM_022997 RefSeq (protein) NP_060676 NP_075373 Location (UCSC) Chr 16: 46.66 – 46.69 Mb Chr 8: 85.99 – 86.03 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Gene In humans, VPS35 is transcribed on chromosome 16q11.2 where is spans about 29.6 kilobases and contains 17 exons. It is ...
Lysosomal storage disorders are caused by lysosomal dysfunction usually as a consequence of deficiency of a single enzyme required for the metabolism of lipids, glycoproteins (sugar-containing proteins), or mucopolysaccharides. Individually, lysosomal storage diseases occur with incidences of less than 1:100,000; however, as a group, the ...
For instance, research with artificial CMA substrate showed that hsc70 chaperone binding to substrate or lysosomal binding does not necessarily require the substrate protein to be capable of unfolding, however, lysosomal translocation makes unfolding as a necessary criteria for it to be internalized. [3]
The gene for LAMP2 has 9 coding exons and 2 alternate last exons, 9a and 9b. [6] When the last exon is spliced with the alternative exon, it is a variant called LAMP2b, which varies in the last 11 amino acids of its C-terminal sequence: in the luminal domain, the transmembrane domain, and the cytoplasmic tail.
Microautophagy is one of the three common forms of autophagic pathway, but unlike macroautophagy and chaperone-mediated autophagy, it is mediated—in mammals by lysosomal action or in plants and fungi by vacuolar action—by direct engulfment of the cytoplasmic cargo.