KEGG   PATHWAY: shr04142
Entry
shr04142                    Pathway                                
Name
Lysosome - Sarcophilus harrisii (Tasmanian devil)
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 catabolism
Pathway map
shr04142  Lysosome
shr04142

Other DBs
GO: 0005764
Organism
Sarcophilus harrisii (Tasmanian devil) [GN:shr]
Gene
100917978  TCIRG1; V-type proton ATPase 116 kDa subunit a [KO:K02154]
105749382  ATP6V0A2; V-type proton ATPase 116 kDa subunit a [KO:K02154]
100934805  ATP6V0A4; V-type proton ATPase 116 kDa subunit a [KO:K02154]
100919520  ATP6V0A1; V-type proton ATPase 116 kDa subunit a isoform X1 [KO:K02154]
100928254  ATP6V0D1; V-type proton ATPase subunit d 1 isoform X1 [KO:K02146]
100913849  ATP6V0D2; V-type proton ATPase subunit d 2 [KO:K02146]
100931636  ATP6V1H; V-type proton ATPase subunit H isoform X1 [KO:K02144]
100924079  ATP6AP1; V-type proton ATPase subunit S1 [KO:K03662]
100924463  ATP6V0C; V-type proton ATPase 16 kDa proteolipid subunit [KO:K02155]
100918992  ATP6V0B; V-type proton ATPase 21 kDa proteolipid subunit [KO:K03661]
100921671  DMXL1; dmX-like protein 1 isoform X1 [KO:K24155]
100935340  DMXL2; dmX-like protein 2 isoform X1 [KO:K24155]
100927741  WDR7; WD repeat-containing protein 7 isoform X1 [KO:K24738]
100918554  NCOA7; nuclear receptor coactivator 7 isoform X1 [KO:K25442]
100933780  CTSA; lysosomal protective protein [KO:K13289] [EC:3.4.16.5]
100915300  lysosomal protective protein-like [KO:K13289] [EC:3.4.16.5]
100920513  CTSB; cathepsin B isoform X1 [KO:K01363] [EC:3.4.22.1]
100914125  CTSC; dipeptidyl peptidase 1 [KO:K01275] [EC:3.4.14.1]
100934264  CTSE; cathepsin E [KO:K01382] [EC:3.4.23.34]
100927375  CTSF; cathepsin F [KO:K01373] [EC:3.4.22.41]
100926518  CTSH; pro-cathepsin H [KO:K01366] [EC:3.4.22.16]
100917440  CTSK; cathepsin K [KO:K01371] [EC:3.4.22.38]
100933802  cathepsin L1 [KO:K01365] [EC:3.4.22.15]
100933548  cathepsin L1 [KO:K01365] [EC:3.4.22.15]
100913383  cathepsin L1-like [KO:K01365] [EC:3.4.22.15]
100928401  CTSO; cathepsin O [KO:K01374] [EC:3.4.22.42]
100917184  CTSS; cathepsin S [KO:K01368] [EC:3.4.22.27]
100929775  CTSW; cathepsin W [KO:K08569] [EC:3.4.22.-]
100927306  CTSZ; cathepsin Z [KO:K08568] [EC:3.4.18.1]
100928633  NAPSA; napsin-A [KO:K08565] [EC:3.4.23.-]
100923637  LGMN; legumain [KO:K01369] [EC:3.4.22.34]
100918462  TPP1; tripeptidyl-peptidase 1 [KO:K01279] [EC:3.4.14.9]
100928928  GLA; alpha-galactosidase A [KO:K01189] [EC:3.2.1.22]
100921364  GLB1; beta-galactosidase isoform X1 [KO:K12309] [EC:3.2.1.23]
100914209  GAA; lysosomal alpha-glucosidase isoform X1 [KO:K12316] [EC:3.2.1.20]
100933026  lysosomal alpha-glucosidase [KO:K12316] [EC:3.2.1.20]
100919688  GBA; lysosomal acid glucosylceramidase [KO:K01201] [EC:3.2.1.45]
100921889  IDUA; alpha-L-iduronidase isoform X1 [KO:K01217] [EC:3.2.1.76]
100925898  NAGA; alpha-N-acetylgalactosaminidase [KO:K01204] [EC:3.2.1.49]
100919780  NAGLU; alpha-N-acetylglucosaminidase [KO:K01205] [EC:3.2.1.50]
100915302  GALC; galactocerebrosidase isoform X1 [KO:K01202] [EC:3.2.1.46]
100915951  GUSB; beta-glucuronidase isoform X1 [KO:K01195] [EC:3.2.1.31]
100919759  tissue alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
100926575  FUCA2; plasma alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
100933252  HEXA; beta-hexosaminidase subunit alpha [KO:K12373] [EC:3.2.1.52]
100919829  HEXB; beta-hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
100915712  MANBA; beta-mannosidase [KO:K01192] [EC:3.2.1.25]
100930413  MAN2B1; lysosomal alpha-mannosidase [KO:K12311] [EC:3.2.1.24]
100930623  NEU1; sialidase-1 isoform X1 [KO:K01186] [EC:3.2.1.18]
100915487  HYAL3; hyaluronidase-3 isoform X1 [KO:K01197] [EC:3.2.1.35]
100916534  HYAL2; hyaluronidase-2 [KO:K01197] [EC:3.2.1.35]
100920054  hyaluronidase-4-like [KO:K01197] [EC:3.2.1.35]
100925107  hyaluronidase-1 [KO:K01197] [EC:3.2.1.35]
100926676  hyaluronidase PH-20 [KO:K01197] [EC:3.2.1.35]
100916007  hyaluronidase-1 [KO:K01197] [EC:3.2.1.35]
100916272  hyaluronidase-1 [KO:K01197] [EC:3.2.1.35]
100930631  ARSA; arylsulfatase A isoform X1 [KO:K01134] [EC:3.1.6.8]
100931900  ARSB; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
100933227  ARSG; arylsulfatase G isoform X1 [KO:K12381] [EC:3.1.6.-]
100933785  GALNS; N-acetylgalactosamine-6-sulfatase isoform X1 [KO:K01132] [EC:3.1.6.4]
100925460  GNS; N-acetylglucosamine-6-sulfatase [KO:K01137] [EC:3.1.6.14]
116422552  N-acetylglucosamine-6-sulfatase-like [KO:K01137] [EC:3.1.6.14]
100932918  IDS; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
100932279  SGSH; N-sulphoglucosamine sulphohydrolase [KO:K01565] [EC:3.10.1.1]
100930405  LIPA; lysosomal acid lipase/cholesteryl ester hydrolase [KO:K01052] [EC:3.1.1.13]
100919470  PLA2G15; group XV phospholipase A2 [KO:K06129] [EC:3.1.1.5]
100927318  DNASE2; deoxyribonuclease-2-alpha isoform X1 [KO:K01158] [EC:3.1.22.1]
100917174  DNASE2B; deoxyribonuclease-2-beta [KO:K01158] [EC:3.1.22.1]
100916495  ACP2; lysosomal acid phosphatase [KO:K14410] [EC:3.1.3.2]
100920615  ACP5; tartrate-resistant acid phosphatase type 5 isoform X1 [KO:K14379] [EC:3.1.3.2]
100916633  SMPD1; sphingomyelin phosphodiesterase [KO:K12350] [EC:3.1.4.12]
100927628  ASAH1; acid ceramidase isoform X1 [KO:K12348] [EC:3.5.1.23]
100930206  AGA; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase [KO:K01444] [EC:3.5.1.26]
100925836  prosaposin [KO:K12382]
105749151  PSAP; prosaposin isoform X1 [KO:K12382]
100920343  GM2A; ganglioside GM2 activator [KO:K12383]
116423468  ganglioside GM2 activator-like [KO:K12383]
100917850  PPT1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
100933490  PPT2; lysosomal thioesterase PPT2 [KO:K01074] [EC:3.1.2.22]
100920201  LAMP1; lysosome-associated membrane glycoprotein 1 [KO:K06528]
100933608  LAMP2; lysosome-associated membrane glycoprotein 2 isoform X2 [KO:K06528]
105750047  LAMP3; lysosome-associated membrane glycoprotein 3 isoform X4 [KO:K06562]
100923517  CD68; macrosialin [KO:K06501]
100921976  CD63; CD63 antigen [KO:K06497]
100924600  SCARB2; lysosome membrane protein 2 [KO:K12384]
100934668  NPC1; NPC intracellular cholesterol transporter 1 isoform X1 [KO:K12385]
100926260  NPC2; NPC intracellular cholesterol transporter 2 [KO:K13443]
100920643  SLC17A5; sialin [KO:K12301]
100920378  SLC11A1; natural resistance-associated macrophage protein 1 [KO:K12347]
100927191  SLC11A2; natural resistance-associated macrophage protein 2 [KO:K21398]
100919588  LAPTM5; lysosomal-associated transmembrane protein 5 [KO:K12387]
100920431  LAPTM4A; lysosomal-associated transmembrane protein 4A [KO:K12387]
100930421  LAPTM4B; lysosomal-associated transmembrane protein 4B [KO:K12387]
100917638  ABCA2; ATP-binding cassette sub-family A member 2 isoform X1 [KO:K05642]
100931729  ABCB9; ATP-binding cassette sub-family B member 9 isoform X1 [KO:K05656]
100919168  CD164; sialomucin core protein 24 isoform X1 [KO:K06546]
100914260  ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 isoform X1 [KO:K12305] [EC:3.6.1.6]
100921440  SORT1; sortilin isoform X1 [KO:K12388]
100917923  CLN3; battenin isoform X1 [KO:K12389]
100930872  CLN5; ceroid-lipofuscinosis neuronal protein 5 [KO:K12390]
100928916  MFSD8; major facilitator superfamily domain-containing protein 8 [KO:K12307]
100930224  HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
100926947  heparan-alpha-glucosaminide N-acetyltransferase-like [KO:K10532] [EC:2.3.1.78]
111719439  SUMF1; formylglycine-generating enzyme isoform X1 [KO:K13444] [EC:1.8.3.7]
100917351  GNPTAB; N-acetylglucosamine-1-phosphotransferase subunits alpha/beta isoform X1 [KO:K08239] [EC:2.7.8.17]
100935201  GNPTG; N-acetylglucosamine-1-phosphotransferase subunit gamma [KO:K10087]
100916276  NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
100926926  IGF2R; cation-independent mannose-6-phosphate receptor [KO:K06564]
100930453  M6PR; cation-dependent mannose-6-phosphate receptor [KO:K10089]
100926895  CLTA; clathrin light chain A isoform X1 [KO:K04644]
100933527  CLTB; clathrin light chain B isoform X2 [KO:K04645]
100920538  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
100935396  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
100925141  AP1G1; AP-1 complex subunit gamma-1 isoform X1 [KO:K12391]
100926953  AP1G2; AP-1 complex subunit gamma-like 2 isoform X1 [KO:K12391]
100927921  AP1B1; AP-1 complex subunit beta-1 isoform X1 [KO:K12392]
100913483  AP1M1; AP-1 complex subunit mu-1 isoform X1 [KO:K12393]
100926975  AP1M2; AP-1 complex subunit mu-2 isoform X2 [KO:K12393]
100914815  AP1S2; AP-1 complex subunit sigma-2 isoform X1 [KO:K12394]
100916131  AP1S1; AP-1 complex subunit sigma-1A isoform X1 [KO:K12394]
100922303  AP1S3; AP-1 complex subunit sigma-3 [KO:K12395]
100918093  AP3D1; AP-3 complex subunit delta-1 isoform X1 [KO:K12396]
100928605  AP3B1; AP-3 complex subunit beta-1 isoform X2 [KO:K12397]
100930208  AP3B2; AP-3 complex subunit beta-2 [KO:K12397]
100916496  AP3M1; AP-3 complex subunit mu-1 [KO:K12398]
100922604  AP3M2; AP-3 complex subunit mu-2 [KO:K12398]
100924103  AP3S1; AP-3 complex subunit sigma-1 isoform X1 [KO:K12399]
100929612  AP-3 complex subunit sigma-2 [KO:K12399]
100934558  AP4E1; AP-4 complex subunit epsilon-1 [KO:K12400]
100921355  AP4B1; AP-4 complex subunit beta-1 [KO:K12401]
100920384  AP4M1; AP-4 complex subunit mu-1 isoform X1 [KO:K12402]
100915198  AP4S1; AP-4 complex subunit sigma-1 [KO:K12403]
100931029  GGA2; ADP-ribosylation factor-binding protein GGA2 [KO:K12404]
100929329  GGA3; ADP-ribosylation factor-binding protein GGA3 isoform X1 [KO:K12404]
100924062  GGA1; ADP-ribosylation factor-binding protein GGA1 [KO:K12404]
100928178  MCOLN1; mucolipin-1 isoform X1 [KO:K04992]
100914625  LITAF; lipopolysaccharide-induced tumor necrosis factor-alpha factor isoform X2 [KO:K19363]
Compound
C00002  ATP
C00008  ADP
C00159  D-Mannose
C00275  D-Mannose 6-phosphate
Reference
  Authors
Eskelinen EL, Tanaka Y, Saftig P
  Title
At the acidic edge: emerging functions for lysosomal membrane proteins.
  Journal
Trends Cell Biol 13:137-45 (2003)
DOI:10.1016/S0962-8924(03)00005-9
Reference
PMID:1883197
  Authors
Neufeld EF
  Title
Lysosomal storage diseases.
  Journal
Annu Rev Biochem 60:257-80 (1991)
DOI:10.1146/annurev.bi.60.070191.001353
Reference
PMID:2943218
  Authors
von Figura K, Hasilik A
  Title
Lysosomal enzymes and their receptors.
  Journal
Annu Rev Biochem 55:167-93 (1986)
DOI:10.1146/annurev.bi.55.070186.001123
Reference
  Authors
Janvier K, Bonifacino JS
  Title
Role of the endocytic machinery in the sorting of lysosome-associated membrane proteins.
  Journal
Mol Biol Cell 16:4231-42 (2005)
DOI:10.1091/mbc.E05-03-0213
Reference
  Authors
Vergarajauregui S, Puertollano R
  Title
Mucolipidosis type IV: the importance of functional lysosomes for efficient autophagy.
  Journal
Autophagy 4:832-4 (2008)
DOI:10.4161/auto.6567
Reference
  Authors
Jaskolka MC, Winkley SR, Kane PM
  Title
RAVE and Rabconnectin-3 Complexes as Signal Dependent Regulators of Organelle Acidification.
  Journal
Front Cell Dev Biol 9:698190 (2021)
DOI:10.3389/fcell.2021.698190
Reference
  Authors
Majdoul S, Compton AA
  Title
Lessons in self-defence: inhibition of virus entry by intrinsic immunity.
  Journal
Nat Rev Immunol 10.1038/s41577-021-00626-8 (2021)
DOI:10.1038/s41577-021-00626-8
Related
pathway
shr00511  Other glycan degradation
shr00531  Glycosaminoglycan degradation
shr04130  SNARE interactions in vesicular transport
shr04140  Autophagy - animal
shr04144  Endocytosis
KO pathway
ko04142   
LinkDB

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