PATHWAY: ko04142 Help
Entry Name Lysosome
Description 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.
Class Cellular Processes; Transport and catabolismBRITE hierarchy
Pathway map Other DBs Orthology K01052 LIPA; lysosomal acid lipase/cholesteryl ester hydrolase [EC:3.1.1.13 ]
K01125 NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [EC:3.1.4.45 ]
K01444 AGA, aspG; N4-(beta-N-acetylglucosaminyl)-L-asparaginase [EC:3.5.1.26 ]
K02144 ATPeV1H; V-type H+-transporting ATPase subunit H
K02146 ATPeV0D, ATP6D; V-type H+-transporting ATPase subunit d
K02154 ATPeV0A, ATP6N; V-type H+-transporting ATPase subunit a
K02155 ATPeV0C, ATP6L; V-type H+-transporting ATPase 16kDa proteolipid subunit
K03661 ATPeV0B, ATP6F; V-type H+-transporting ATPase 21kDa proteolipid subunit
K03662 ATPeVS1, ATP6S1; V-type H+-transporting ATPase S1 subunit
K04644 CLTA, LCA; clathrin light chain A
K04645 CLTB, LCB; clathrin light chain B
K04646 CLTC; clathrin heavy chain
K04992 MCOLN1, TRPML1; mucolipin 1
K05642 ABCA2; ATP-binding cassette, subfamily A (ABC1), member 2
K05656 ABCB9, TAPL; ATP-binding cassette, subfamily B (MDR/TAP), member 9
K06497 CD63, MLA1, TSPAN30; CD63 antigen
K06528 LAMP1_2, CD107; lysosomal-associated membrane protein 1/2
K06546 CD164, MGC24; CD164 antigen
K06562 LAMP3, CD208; lysosomal-associated membrane protein 3
K06564 IGF2R, CD222; insulin-like growth factor 2 receptor
K08565 NAPSA; napsin-A [EC:3.4.23.-]
K08569 CTSW; cathepsin W [EC:3.4.22.-]
K09599 CTSP; cathepsin P [EC:3.4.22.-]
K09600 CTSM; cathepsin M [EC:3.4.22.-]
K10087 GNPTG, GNPTAG; N-acetylglucosamine-1-phosphate transferase, gamma subunit
K10089 M6PR; cation-dependent mannose-6-phosphate receptor
K10532 HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase [EC:2.3.1.78 ]
K12301 SLC17A5; MFS transporter, ACS family, solute carrier family 17 (sodium-dependent inorganic phosphate cotransporter), member 5
K12305 ENTPD4, LALP70; ectonucleoside triphosphate diphosphohydrolase 4 [EC:3.6.1.6 ]
K12307 MSFD8, CLN7; MFS transporter, DHA1 family, ceroid-lipofuscinosis neuronal protein 7
K12347 SLC11A1, NRAMP1; natural resistance-associated macrophage protein 1
K12381 ARSG; arylsulfatase G [EC:3.1.6.-]
K12383 GM2A; ganglioside GM2 activator
K12384 SCARB2, LIMP2, CD36L2; lysosome membrane protein 2
K12385 NPC1; Niemann-Pick C1 protein
K12387 LAPTM; lysosomal-associated transmembrane protein
K12390 CLN5; ceroid-lipofuscinosis neuronal protein 5
K12391 AP1G1; AP-1 complex subunit gamma-1
K12392 AP1B1; AP-1 complex subunit beta-1
K12393 AP1M; AP-1 complex subunit mu
K12394 AP1S1_2; AP-1 complex subunit sigma 1/2
K12395 AP1S3; AP-1 complex subunit sigma-3
K12396 AP3D; AP-3 complex subunit delta
K12397 AP3B; AP-3 complex subunit beta
K12398 AP3M; AP-3 complex subunit mu
K12399 AP3S; AP-3 complex subunit sigma
K12400 AP4E1; AP-4 complex subunit epsilon-1
K12401 AP4B1; AP-4 complex subunit beta-1
K12402 AP4M1; AP-4 complex subunit mu-1
K12403 AP4S1; AP-4 complex subunit sigma-1
K12404 GGA; ADP-ribosylation factor-binding protein GGA
K13443 NPC2; Niemann-Pick C2 protein
K14379 ACP5; tartrate-resistant acid phosphatase type 5 [EC:3.1.3.2 ]
K19363 LITAF; lipopolysaccharide-induced tumor necrosis factor-alpha factor
K21398 SLC11A2, DMT1, NRAMP2; natural resistance-associated macrophage protein 2
K24155 DMXL, DMX, RAV1; rabconnectin-3a
K25442 NCOA7; nuclear receptor coactivator 7
K27947 MEAK7, TLDC1; MTOR-associated protein MEAK7
Compound Reference Authors Eskelinen EL, Tanaka Y, Saftig P
Title At the acidic edge: emerging functions for lysosomal membrane proteins.
Journal Reference Authors Neufeld EF
Title Lysosomal storage diseases.
Journal Reference Authors von Figura K, Hasilik A
Title Lysosomal enzymes and their receptors.
Journal Reference Authors Janvier K, Bonifacino JS
Title Role of the endocytic machinery in the sorting of lysosome-associated membrane proteins.
Journal Reference Authors Vergarajauregui S, Puertollano R
Title Mucolipidosis type IV: the importance of functional lysosomes for efficient autophagy.
Journal Reference Authors Jaskolka MC, Winkley SR, Kane PM
Title RAVE and Rabconnectin-3 Complexes as Signal Dependent Regulators of Organelle Acidification.
Journal Reference Authors Majdoul S, Compton AA
Title Lessons in self-defence: inhibition of virus entry by intrinsic immunity.
Journal Related ko00531 Glycosaminoglycan degradation
ko04130 SNARE interactions in vesicular transport
