Lysosomes are membrane-delimited organelles in animal cells serving as the cell's main digestive compartment to which all sorts of macromolecules are delivered for degradation. They contain more than 40 hydrolases in an acidic environment (pH of about 5). After synthesis in the ER, lysosomal enzymes are decorated with mannose-6-phosphate residues, which are recognized by mannose-6-phosphate receptors in the trans-Golgi network. They are packaged into clathrin-coated vesicles and are transported to late endosomes. Substances for digestion are acquired by the lysosomes via a series of processes including endocytosis, phagocytosis, and autophagy.
Cellular Processes; Transport and catabolism
Pathway map Ortholog table
K02154 V-type H+-transporting ATPase subunit a
K02146 V-type H+-transporting ATPase subunit d
K02144 V-type H+-transporting ATPase subunit H
K03662 V-type H+-transporting ATPase S1 subunit
K02155 V-type H+-transporting ATPase 16kDa proteolipid subunit
K03661 V-type H+-transporting ATPase 21kDa proteolipid subunit
K25442 nuclear receptor coactivator 7
K09599 cathepsin P [EC:3.4.22.-]
K09600 cathepsin M [EC:3.4.22.-]
K08569 cathepsin W [EC:3.4.22.-]
K12381 arylsulfatase G [EC:3.1.6.-]
K01052 lysosomal acid lipase/cholesteryl ester hydrolase [EC: 184.108.40.206]
K12383 ganglioside GM2 activator
K06528 lysosomal-associated membrane protein 1/2
K06562 lysosomal-associated membrane protein 3
K12384 lysosome membrane protein 2
K12385 Niemann-Pick C1 protein
K13443 Niemann-Pick C2 protein
K12301 MFS transporter, ACS family, solute carrier family 17 (sodium-dependent inorganic phosphate cotransporter), member 5
K12347 natural resistance-associated macrophage protein 1
K21398 natural resistance-associated macrophage protein 2
K12387 lysosomal-associated transmembrane protein
K05642 ATP-binding cassette, subfamily A (ABC1), member 2
K05656 ATP-binding cassette, subfamily B (MDR/TAP), member 9
K12305 ectonucleoside triphosphate diphosphohydrolase 4 [EC: 220.127.116.11]
K12390 ceroid-lipofuscinosis neuronal protein 5
K12307 MFS transporter, ceroid-lipofuscinosis neuronal protein 7
K10087 N-acetylglucosamine-1-phosphate transferase, gamma subunit
K01125 N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [EC: 18.104.22.168]
K06564 insulin-like growth factor 2 receptor
K10089 cation-dependent mannose-6-phosphate receptor
K12391 AP-1 complex subunit gamma-1
K12392 AP-1 complex subunit beta-1
K12393 AP-1 complex subunit mu
K12394 AP-1 complex subunit sigma 1/2
K12395 AP-1 complex subunit sigma-3
K12396 AP-3 complex subunit delta
K12397 AP-3 complex subunit beta
K12398 AP-3 complex subunit mu
K12399 AP-3 complex subunit sigma
K12400 AP-4 complex subunit epsilon-1
K12401 AP-4 complex subunit beta-1
K12402 AP-4 complex subunit mu-1
K12403 AP-4 complex subunit sigma-1
K12404 ADP-ribosylation factor-binding protein GGA
K19363 lipopolysaccharide-induced tumor necrosis factor-alpha factor
Eskelinen EL, Tanaka Y, Saftig P
At the acidic edge: emerging functions for lysosomal membrane proteins.
Lysosomal storage diseases.
von Figura K, Hasilik A
Lysosomal enzymes and their receptors.
Janvier K, Bonifacino JS
Role of the endocytic machinery in the sorting of lysosome-associated membrane proteins.
Vergarajauregui S, Puertollano R
Mucolipidosis type IV: the importance of functional lysosomes for efficient autophagy.
Jaskolka MC, Winkley SR, Kane PM
RAVE and Rabconnectin-3 Complexes as Signal Dependent Regulators of Organelle Acidification.
Majdoul S, Compton AA
Lessons in self-defence: inhibition of virus entry by intrinsic immunity.
ko00531 Glycosaminoglycan degradation
ko04130 SNARE interactions in vesicular transport
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