KEGG   PATHWAY: ko04142
ko04142                     Pathway                                
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
ko04142  Lysosome

Other DBs
GO: 0005764
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
K24155  rabconnectin-3a
K24738  rabconnectin-3b
K25442  nuclear receptor coactivator 7
K13289  cathepsin A (carboxypeptidase C) [EC:]
K01363  cathepsin B [EC:]
K01275  cathepsin C [EC:]
K01379  cathepsin D [EC:]
K01382  cathepsin E [EC:]
K01373  cathepsin F [EC:]
K01319  cathepsin G [EC:]
K01366  cathepsin H [EC:]
K09599  cathepsin P [EC:3.4.22.-]
K01371  cathepsin K [EC:]
K01365  cathepsin L [EC:]
K09600  cathepsin M [EC:3.4.22.-]
K01374  cathepsin O [EC:]
K01368  cathepsin S [EC:]
K01375  cathepsin V [EC:]
K08569  cathepsin W [EC:3.4.22.-]
K08568  cathepsin X [EC:]
K08565  napsin-A [EC:3.4.23.-]
K01369  legumain [EC:]
K01279  tripeptidyl-peptidase I [EC:]
K01189  alpha-galactosidase [EC:]
K12309  beta-galactosidase [EC:]
K12316  lysosomal alpha-glucosidase [EC:]
K01201  glucosylceramidase [EC:]
K01217  L-iduronidase [EC:]
K01204  alpha-N-acetylgalactosaminidase [EC:]
K01205  alpha-N-acetylglucosaminidase [EC:]
K01202  galactosylceramidase [EC:]
K01195  beta-glucuronidase [EC:]
K01206  alpha-L-fucosidase [EC:]
K12373  hexosaminidase [EC:]
K01192  beta-mannosidase [EC:]
K12311  lysosomal alpha-mannosidase [EC:]
K01186  sialidase-1 [EC:]
K01197  hyaluronoglucosaminidase [EC:]
K01134  arylsulfatase A [EC:]
K01135  arylsulfatase B [EC:]
K12381  arylsulfatase G [EC:3.1.6.-]
K01132  N-acetylgalactosamine-6-sulfatase [EC:]
K01137  N-acetylglucosamine-6-sulfatase [EC:]
K01136  iduronate 2-sulfatase [EC:]
K01565  N-sulfoglucosamine sulfohydrolase [EC:]
K01052  lysosomal acid lipase/cholesteryl ester hydrolase [EC:]
K06129  lysophospholipase III [EC:]
K01158  deoxyribonuclease II [EC:]
K14410  lysosomal acid phosphatase [EC:]
K14379  tartrate-resistant acid phosphatase type 5 [EC:]
K12350  sphingomyelin phosphodiesterase [EC:]
K12348  acid ceramidase [EC:]
K01444  N4-(beta-N-acetylglucosaminyl)-L-asparaginase [EC:]
K12382  saposin
K12383  ganglioside GM2 activator
K01074  palmitoyl-protein thioesterase [EC:]
K06528  lysosomal-associated membrane protein 1/2
K06562  lysosomal-associated membrane protein 3
K06501  CD68 antigen
K06497  CD63 antigen
K12384  lysosome membrane protein 2
K12385  Niemann-Pick C1 protein
K13443  Niemann-Pick C2 protein
K12386  cystinosin
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
K06546  CD164 antigen
K12305  ectonucleoside triphosphate diphosphohydrolase 4 [EC:]
K12388  sortilin
K12389  battenin
K12390  ceroid-lipofuscinosis neuronal protein 5
K12307  MFS transporter, ceroid-lipofuscinosis neuronal protein 7
K10532  heparan-alpha-glucosaminide N-acetyltransferase [EC:]
K13444  formylglycine-generating enzyme [EC:]
K08239  UDP-N-acetylglucosamine-lysosomal-enzyme [EC:]
K10087  N-acetylglucosamine-1-phosphate transferase, gamma subunit
K01125  N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [EC:]
K06564  insulin-like growth factor 2 receptor
K10089  cation-dependent mannose-6-phosphate receptor
K04644  clathrin light chain A
K04645  clathrin light chain B
K04646  clathrin heavy chain
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
K04992  mucolipin 1
K19363  lipopolysaccharide-induced tumor necrosis factor-alpha factor
C00002  ATP
C00008  ADP
C00159  D-Mannose
C00275  D-Mannose 6-phosphate
Eskelinen EL, Tanaka Y, Saftig P
At the acidic edge: emerging functions for lysosomal membrane proteins.
Trends Cell Biol 13:137-45 (2003)
Neufeld EF
Lysosomal storage diseases.
Annu Rev Biochem 60:257-80 (1991)
von Figura K, Hasilik A
Lysosomal enzymes and their receptors.
Annu Rev Biochem 55:167-93 (1986)
Janvier K, Bonifacino JS
Role of the endocytic machinery in the sorting of lysosome-associated membrane proteins.
Mol Biol Cell 16:4231-42 (2005)
Vergarajauregui S, Puertollano R
Mucolipidosis type IV: the importance of functional lysosomes for efficient autophagy.
Autophagy 4:832-4 (2008)
Jaskolka MC, Winkley SR, Kane PM
RAVE and Rabconnectin-3 Complexes as Signal Dependent Regulators of Organelle Acidification.
Front Cell Dev Biol 9:698190 (2021)
Majdoul S, Compton AA
Lessons in self-defence: inhibition of virus entry by intrinsic immunity.
Nat Rev Immunol 10.1038/s41577-021-00626-8 (2021)
ko00511  Other glycan degradation
ko00531  Glycosaminoglycan degradation
ko04130  SNARE interactions in vesicular transport
ko04140  Autophagy - animal
ko04144  Endocytosis

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