KEGG   PATHWAY: pcoq04142
Entry
pcoq04142                   Pathway                                
Name
Lysosome - Propithecus coquereli (Coquerel's sifaka)
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
pcoq04142  Lysosome
pcoq04142

Other DBs
GO: 0005764
Organism
Propithecus coquereli (Coquerel's sifaka) [GN:pcoq]
Gene
105825687  ATP6V0A2; V-type proton ATPase 116 kDa subunit a isoform 2 [KO:K02154]
105820210  ATP6V0A1; V-type proton ATPase 116 kDa subunit a isoform 1 [KO:K02154]
105827412  TCIRG1; V-type proton ATPase 116 kDa subunit a isoform 3 isoform X1 [KO:K02154]
105809009  ATP6V0A4; V-type proton ATPase 116 kDa subunit a isoform 4 [KO:K02154]
105823367  ATP6V0D1; V-type proton ATPase subunit d 1 [KO:K02146]
105809748  ATP6V0D2; V-type proton ATPase subunit d 2 [KO:K02146]
105824057  ATP6V1H; V-type proton ATPase subunit H [KO:K02144]
105824521  ATP6AP1; V-type proton ATPase subunit S1 [KO:K03662]
105813115  ATP6V0C; V-type proton ATPase 16 kDa proteolipid subunit [KO:K02155]
105814097  ATP6V0B; V-type proton ATPase 21 kDa proteolipid subunit [KO:K03661]
105821326  DMXL2; dmX-like protein 2 [KO:K24155]
105822555  DMXL1; dmX-like protein 1 isoform X1 [KO:K24155]
105816387  WDR7; WD repeat-containing protein 7 [KO:K24738]
105823551  NCOA7; nuclear receptor coactivator 7 [KO:K25442]
105807161  CTSA; lysosomal protective protein [KO:K13289] [EC:3.4.16.5]
105811637  CTSB; cathepsin B [KO:K01363] [EC:3.4.22.1]
105808237  CTSC; dipeptidyl peptidase 1 isoform X1 [KO:K01275] [EC:3.4.14.1]
105816553  CTSD; cathepsin D [KO:K01379] [EC:3.4.23.5]
105821191  CTSE; cathepsin E isoform X1 [KO:K01382] [EC:3.4.23.34]
105827466  CTSF; cathepsin F [KO:K01373] [EC:3.4.22.41]
105812758  CTSG; cathepsin G [KO:K01319] [EC:3.4.21.20]
105822761  CTSH; pro-cathepsin H [KO:K01366] [EC:3.4.22.16]
105821962  CTSK; cathepsin K [KO:K01371] [EC:3.4.22.38]
105822404  CTSL; cathepsin L1 [KO:K01365] [EC:3.4.22.15]
105816430  CTSV; cathepsin L2 [KO:K01365] [EC:3.4.22.15]
105811126  CTSO; cathepsin O [KO:K01374] [EC:3.4.22.42]
105821963  CTSS; cathepsin S [KO:K01368] [EC:3.4.22.27]
105827350  CTSW; cathepsin W [KO:K08569] [EC:3.4.22.-]
105820086  CTSZ; cathepsin Z [KO:K08568] [EC:3.4.18.1]
105809341  NAPSA; napsin-A [KO:K08565] [EC:3.4.23.-]
105820278  LGMN; legumain [KO:K01369] [EC:3.4.22.34]
105818381  TPP1; tripeptidyl-peptidase 1 [KO:K01279] [EC:3.4.14.9]
105814939  GLA; alpha-galactosidase A [KO:K01189] [EC:3.2.1.22]
105812427  GLB1; beta-galactosidase [KO:K12309] [EC:3.2.1.23]
105825318  GAA; lysosomal alpha-glucosidase [KO:K12316] [EC:3.2.1.20]
105821903  GBA; glucosylceramidase [KO:K01201] [EC:3.2.1.45]
105825802  IDUA; alpha-L-iduronidase [KO:K01217] [EC:3.2.1.76]
105805270  NAGA; alpha-N-acetylgalactosaminidase isoform X1 [KO:K01204] [EC:3.2.1.49]
105820236  NAGLU; alpha-N-acetylglucosaminidase [KO:K01205] [EC:3.2.1.50]
105807113  GALC; LOW QUALITY PROTEIN: galactocerebrosidase [KO:K01202] [EC:3.2.1.46]
105807071  GUSB; beta-glucuronidase isoform X1 [KO:K01195] [EC:3.2.1.31]
105819321  FUCA2; plasma alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
105815682  FUCA1; LOW QUALITY PROTEIN: tissue alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
105826593  HEXB; beta-hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
105807236  HEXA; beta-hexosaminidase subunit alpha [KO:K12373] [EC:3.2.1.52]
105819185  MANBA; beta-mannosidase [KO:K01192] [EC:3.2.1.25]
105807911  MAN2B1; lysosomal alpha-mannosidase [KO:K12311] [EC:3.2.1.24]
105817874  NEU1; sialidase-1 [KO:K01186] [EC:3.2.1.18]
105821648  HYAL3; hyaluronidase-3 [KO:K01197] [EC:3.2.1.35]
105805222  HYAL2; hyaluronidase-2 [KO:K01197] [EC:3.2.1.35]
105817097  hyaluronidase PH-20-like [KO:K01197] [EC:3.2.1.35]
105817098  hyaluronidase PH-20-like [KO:K01197] [EC:3.2.1.35]
105817101  hyaluronidase PH-20-like [KO:K01197] [EC:3.2.1.35]
105817142  HYAL4; hyaluronidase-4 [KO:K01197] [EC:3.2.1.35]
105806003  HYAL1; hyaluronidase-1 isoform X1 [KO:K01197] [EC:3.2.1.35]
105813956  ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
105826553  ARSB; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
105824728  ARSG; arylsulfatase G [KO:K12381] [EC:3.1.6.-]
105821060  GALNS; N-acetylgalactosamine-6-sulfatase [KO:K01132] [EC:3.1.6.4]
105824197  GNS; N-acetylglucosamine-6-sulfatase [KO:K01137] [EC:3.1.6.14]
105827243  IDS; iduronate 2-sulfatase isoform X1 [KO:K01136] [EC:3.1.6.13]
105825321  SGSH; N-sulphoglucosamine sulphohydrolase [KO:K01565] [EC:3.10.1.1]
105822717  LIPA; lysosomal acid lipase/cholesteryl ester hydrolase [KO:K01052] [EC:3.1.1.13]
105823418  PLA2G15; group XV phospholipase A2 [KO:K06129] [EC:3.1.1.5]
105823818  DNASE2B; deoxyribonuclease-2-beta [KO:K01158] [EC:3.1.22.1]
105807868  DNASE2; deoxyribonuclease-2-alpha [KO:K01158] [EC:3.1.22.1]
105811077  ACP2; lysosomal acid phosphatase [KO:K14410] [EC:3.1.3.2]
105807836  ACP5; tartrate-resistant acid phosphatase type 5 [KO:K14379] [EC:3.1.3.2]
105818403  SMPD1; sphingomyelin phosphodiesterase [KO:K12350] [EC:3.1.4.12]
105825204  ASAH1; acid ceramidase [KO:K12348] [EC:3.5.1.23]
105816278  AGA; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase isoform X1 [KO:K01444] [EC:3.5.1.26]
105825471  PSAP; prosaposin isoform X1 [KO:K12382]
105820284  PSAPL1; proactivator polypeptide-like 1 [KO:K12382]
105821549  GM2A; ganglioside GM2 activator [KO:K12383]
105817855  PPT2; lysosomal thioesterase PPT2 isoform X1 [KO:K01074] [EC:3.1.2.22]
105812301  PPT1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
105825142  LAMP1; lysosome-associated membrane glycoprotein 1 [KO:K06528]
105825169  LAMP2; lysosome-associated membrane glycoprotein 2 isoform X1 [KO:K06528]
105807415  LAMP3; lysosome-associated membrane glycoprotein 3 [KO:K06562]
105819403  CD68; macrosialin [KO:K06501]
105817209  CD63; CD63 antigen [KO:K06497]
105815747  SCARB2; lysosome membrane protein 2 [KO:K12384]
105825017  NPC1; Niemann-Pick C1 protein [KO:K12385]
105819882  NPC2; epididymal secretory protein E1 [KO:K13443]
105817006  CTNS; cystinosin isoform X1 [KO:K12386]
105811776  SLC17A5; sialin [KO:K12301]
105808823  SLC11A1; natural resistance-associated macrophage protein 1 isoform X1 [KO:K12347]
105817748  SLC11A2; natural resistance-associated macrophage protein 2 isoform X1 [KO:K21398]
105815021  natural resistance-associated macrophage protein 2-like [KO:K21398]
105808647  LAPTM5; lysosomal-associated transmembrane protein 5 [KO:K12387]
105809770  LAPTM4B; lysosomal-associated transmembrane protein 4B [KO:K12387]
105817407  LAPTM4A; lysosomal-associated transmembrane protein 4A [KO:K12387]
105818710  ABCA2; ATP-binding cassette sub-family A member 2 [KO:K05642]
105825684  ABCB9; ATP-binding cassette sub-family B member 9 isoform X1 [KO:K05656]
105819877  LOW QUALITY PROTEIN: sialomucin core protein 24-like [KO:K06546]
105815875  CD164; sialomucin core protein 24 [KO:K06546]
105825271  ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 isoform X1 [KO:K12305] [EC:3.6.1.6]
105813755  SORT1; sortilin isoform X1 [KO:K12388]
105823178  CLN3; battenin isoform X1 [KO:K12389]
105804994  CLN5; ceroid-lipofuscinosis neuronal protein 5 [KO:K12390]
105809479  MFSD8; major facilitator superfamily domain-containing protein 8 [KO:K12307]
105819694  HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
105812727  SUMF1; sulfatase-modifying factor 1 [KO:K13444] [EC:1.8.3.7]
105815137  GNPTAB; N-acetylglucosamine-1-phosphotransferase subunits alpha/beta [KO:K08239] [EC:2.7.8.17]
105813082  GNPTG; N-acetylglucosamine-1-phosphotransferase subunit gamma isoform X1 [KO:K10087]
105813174  NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
105812399  IGF2R; LOW QUALITY PROTEIN: cation-independent mannose-6-phosphate receptor [KO:K06564]
105805075  M6PR; cation-dependent mannose-6-phosphate receptor isoform X1 [KO:K10089]
105820126  CLTA; clathrin light chain A [KO:K04644]
105822510  CLTB; clathrin light chain B [KO:K04645]
105818837  CLTCL1; LOW QUALITY PROTEIN: clathrin heavy chain 2 [KO:K04646]
105813663  CLTC; clathrin heavy chain 1 [KO:K04646]
105823388  AP1G1; AP-1 complex subunit gamma-1 isoform X1 [KO:K12391]
105814438  AP1G2; AP-1 complex subunit gamma-like 2 [KO:K12391]
105810213  AP1B1; AP-1 complex subunit beta-1 isoform X1 [KO:K12392]
105825702  AP1M1; AP-1 complex subunit mu-1 [KO:K12393]
105807829  AP1M2; AP-1 complex subunit mu-2 isoform X1 [KO:K12393]
105826031  AP1S1; AP-1 complex subunit sigma-1A [KO:K12394]
105805772  AP1S2; AP-1 complex subunit sigma-2 isoform X1 [KO:K12394]
105808832  AP1S3; AP-1 complex subunit sigma-3 [KO:K12395]
105811275  AP3D1; AP-3 complex subunit delta-1 [KO:K12396]
105820107  AP3B2; AP-3 complex subunit beta-2 isoform X1 [KO:K12397]
105826499  AP3B1; AP-3 complex subunit beta-1 [KO:K12397]
105819670  AP3M2; AP-3 complex subunit mu-2 [KO:K12398]
105825504  AP3M1; AP-3 complex subunit mu-1 [KO:K12398]
105822544  AP3S1; AP-3 complex subunit sigma-1 [KO:K12399]
105809875  AP3S2; AP-3 complex subunit sigma-2 [KO:K12399]
105814262  AP4E1; AP-4 complex subunit epsilon-1 [KO:K12400]
105810604  AP4B1; AP-4 complex subunit beta-1 isoform X1 [KO:K12401]
105805984  AP4M1; AP-4 complex subunit mu-1 [KO:K12402]
105813243  AP4S1; AP-4 complex subunit sigma-1 [KO:K12403]
105824749  GGA3; ADP-ribosylation factor-binding protein GGA3 [KO:K12404]
105810888  GGA2; ADP-ribosylation factor-binding protein GGA2 [KO:K12404]
105805197  GGA1; ADP-ribosylation factor-binding protein GGA1 [KO:K12404]
105807927  MCOLN1; mucolipin-1 [KO:K04992]
105813098  LITAF; lipopolysaccharide-induced tumor necrosis factor-alpha factor [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
pcoq00511  Other glycan degradation
pcoq00531  Glycosaminoglycan degradation
pcoq04130  SNARE interactions in vesicular transport
pcoq04140  Autophagy - animal
pcoq04144  Endocytosis
KO pathway
ko04142   
LinkDB

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