KEGG   PATHWAY: rbb04142
Entry
rbb04142                    Pathway                                
Name
Lysosome - Rhinopithecus bieti (black snub-nosed monkey)
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
rbb04142  Lysosome
rbb04142

Other DBs
GO: 0005764
Organism
Rhinopithecus bieti (black snub-nosed monkey) [GN:rbb]
Gene
108512374  FUCA2; LOW QUALITY PROTEIN: plasma alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
108512818  GGA1; ADP-ribosylation factor-binding protein GGA1 isoform X1 [KO:K12404]
108514204  AP1S3; AP-1 complex subunit sigma-3 [KO:K12395]
108514313  CD68; macrosialin [KO:K06501]
108515379  PLA2G15; group XV phospholipase A2 isoform X1 [KO:K06129] [EC:3.1.1.5]
108515413  ATP6V0D1; V-type proton ATPase subunit d 1 isoform X1 [KO:K02146]
108516031  CD63; CD63 antigen [KO:K06497]
108516095  PSAPL1; proactivator polypeptide-like 1 [KO:K12382]
108516180  IGF2R; cation-independent mannose-6-phosphate receptor isoform X1 [KO:K06564]
108516648  CLN5; ceroid-lipofuscinosis neuronal protein 5 [KO:K12390]
108516862  PSAP; prosaposin isoform X1 [KO:K12382]
108516868  AP3M1; AP-3 complex subunit mu-1 [KO:K12398]
108517399  ATP6V1H; V-type proton ATPase subunit H isoform X1 [KO:K02144]
108517586  AP1B1; LOW QUALITY PROTEIN: AP-1 complex subunit beta-1 [KO:K12392]
108518317  ATP6V0A1; V-type proton ATPase 116 kDa subunit a [KO:K02154]
108518334  NAGLU; alpha-N-acetylglucosaminidase [KO:K01205] [EC:3.2.1.50]
108518391  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
108518407  SLC11A1; natural resistance-associated macrophage protein 1 [KO:K12347]
108518589  LAPTM5; lysosomal-associated transmembrane protein 5 [KO:K12387]
108518640  CTSW; cathepsin W [KO:K08569] [EC:3.4.22.-]
108518671  CTSF; LOW QUALITY PROTEIN: cathepsin F [KO:K01373] [EC:3.4.22.41]
108518675  TCIRG1; V-type proton ATPase 116 kDa subunit a isoform X1 [KO:K02154]
108518773  ATP6AP1; V-type proton ATPase subunit S1 [KO:K03662]
108518942  AP1S1; AP-1 complex subunit sigma-1A [KO:K12394]
108519101  LGMN; legumain [KO:K01369] [EC:3.4.22.34]
108519424  GNPTG; N-acetylglucosamine-1-phosphotransferase subunit gamma [KO:K10087]
108519445  ATP6V0C; V-type proton ATPase 16 kDa proteolipid subunit [KO:K02155]
108519634  SORT1; LOW QUALITY PROTEIN: sortilin [KO:K12388]
108519890  PPT2; lysosomal thioesterase PPT2 isoform X1 [KO:K01074] [EC:3.1.2.22]
108519980  GALNS; N-acetylgalactosamine-6-sulfatase [KO:K01132] [EC:3.1.6.4]
108520269  NEU1; sialidase-1 isoform X1 [KO:K01186] [EC:3.2.1.18]
108521063  LAMP3; lysosome-associated membrane glycoprotein 3 isoform X1 [KO:K06562]
108521125  TLDC1; TLD domain-containing protein 1 [KO:K27947]
108521151  [KO:K01365] [EC:3.4.22.15]
108521156  CTSL; cathepsin L1 [KO:K01365] [EC:3.4.22.15]
108521374  GLA; alpha-galactosidase A [KO:K01189] [EC:3.2.1.22]
108521411  AP3S2; AP-3 complex subunit sigma-2 [KO:K12399]
108521603  SLC11A2; natural resistance-associated macrophage protein 2 isoform X1 [KO:K21398]
108521829  LAMP1; lysosome-associated membrane glycoprotein 1 [KO:K06528]
108521963  ACP2; lysosomal acid phosphatase isoform X1 [KO:K14410] [EC:3.1.3.2]
108522658  NCOA7; nuclear receptor coactivator 7 isoform X1 [KO:K25442]
108522791  GALC; galactocerebrosidase [KO:K01202] [EC:3.2.1.46]
108522825  LAMP2; LOW QUALITY PROTEIN: lysosome-associated membrane glycoprotein 2 [KO:K06528]
108523018  ARSG; arylsulfatase G isoform X1 [KO:K12381] [EC:3.1.6.-]
108523097  CTNS; cystinosin isoform X1 [KO:K12386]
108523206  ATP6V0D2; V-type proton ATPase subunit d 2 [KO:K02146]
108523239  SPAM1; hyaluronidase PH-20 [KO:K01197] [EC:3.2.1.35]
108523241  [KO:K01197] [EC:3.2.1.35]
108523242  HYAL4; hyaluronidase-4 [KO:K01197] [EC:3.2.1.35]
108523589  AP1G2; AP-1 complex subunit gamma-like 2 isoform X1 [KO:K12391]
108523674  CLN3; battenin isoform X1 [KO:K12389]
108524364  IDUA; alpha-L-iduronidase [KO:K01217] [EC:3.2.1.76]
108524797  [KO:K02155]
108525811  [KO:K01134] [EC:3.1.6.8]
108526218  [KO:K12383]
108526474  ASAH1; acid ceramidase [KO:K12348] [EC:3.5.1.23]
108526597  LIPA; lysosomal acid lipase/cholesteryl ester hydrolase [KO:K01052] [EC:3.1.1.13]
108526954  HYAL2; LOW QUALITY PROTEIN: hyaluronidase-2 [KO:K01197] [EC:3.2.1.35]
108526956  HYAL1; hyaluronidase-1 isoform X1 [KO:K01197] [EC:3.2.1.35]
108526957  HYAL3; hyaluronidase-3 [KO:K01197] [EC:3.2.1.35]
108527464  AP1G1; AP-1 complex subunit gamma-1 isoform X1 [KO:K12391]
108527686  DNASE2B; deoxyribonuclease-2-beta isoform X1 [KO:K01158] [EC:3.1.22.1]
108527907  ACP5; tartrate-resistant acid phosphatase type 5 isoform X1 [KO:K14379] [EC:3.1.3.2]
108527997  GUSB; LOW QUALITY PROTEIN: beta-glucuronidase [KO:K01195] [EC:3.2.1.31]
108528111  AP4B1; AP-4 complex subunit beta-1 isoform X1 [KO:K12401]
108528742  SUMF1; sulfatase-modifying factor 1 isoform X1 [KO:K13444] [EC:1.8.3.7]
108528898  ARSB; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
108529021  CTSO; LOW QUALITY PROTEIN: cathepsin O [KO:K01374] [EC:3.4.22.42]
108529193  SLC17A5; sialin isoform X1 [KO:K12301]
108529277  CTSV; cathepsin L2 [KO:K01375] [EC:3.4.22.43]
108529494  CTSG; cathepsin G [KO:K01319] [EC:3.4.21.20]
108529831  DMXL1; LOW QUALITY PROTEIN: dmX-like protein 1 [KO:K24155]
108529983  GNS; N-acetylglucosamine-6-sulfatase isoform X1 [KO:K01137] [EC:3.1.6.14]
108530410  PPT1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
108531139  [KO:K19363]
108531312  CTSD; cathepsin D [KO:K01379] [EC:3.4.23.5]
108531436  AP3D1; AP-3 complex subunit delta-1 isoform X1 [KO:K12396]
108531718  LAPTM4B; LOW QUALITY PROTEIN: lysosomal-associated transmembrane protein 4B [KO:K12387]
108532029  GM2A; ganglioside GM2 activator [KO:K12383]
108532486  SCARB2; lysosome membrane protein 2 [KO:K12384]
108532812  MANBA; beta-mannosidase isoform X1 [KO:K01192] [EC:3.2.1.25]
108533052  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
108533261  CTSK; cathepsin K [KO:K01371] [EC:3.4.22.38]
108533262  CTSS; cathepsin S [KO:K01368] [EC:3.4.22.27]
108533329  CTSA; lysosomal protective protein isoform X1 [KO:K13289] [EC:3.4.16.5]
108533491  [KO:K02155]
108533804  CD164; sialomucin core protein 24 isoform X1 [KO:K06546]
108534035  AGA; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase isoform X1 [KO:K01444] [EC:3.5.1.26]
108534150  SGSH; N-sulphoglucosamine sulphohydrolase [KO:K01565] [EC:3.10.1.1]
108534154  GAA; lysosomal alpha-glucosidase isoform X1 [KO:K12316] [EC:3.2.1.20]
108534354  HGSNAT; LOW QUALITY PROTEIN: heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
108534588  MCOLN1; LOW QUALITY PROTEIN: mucolipin-1 [KO:K04992]
108534827  ATP6V0A4; V-type proton ATPase 116 kDa subunit a isoform X1 [KO:K02154]
108534912  NPC2; epididymal secretory protein E1 isoform X1 [KO:K13443]
108535115  CTSE; cathepsin E [KO:K01382] [EC:3.4.23.34]
108535688  ATP6V0B; V-type proton ATPase 21 kDa proteolipid subunit isoform X1 [KO:K03661]
108535801  NAPSA; napsin-A [KO:K08565] [EC:3.4.23.-]
108535802  [KO:K08565] [EC:3.4.23.-]
108536689  CTSB; cathepsin B isoform X1 [KO:K01363] [EC:3.4.22.1]
108536911  NPC1; Niemann-Pick C1 protein isoform X1 [KO:K12385]
108537399  GGA3; ADP-ribosylation factor-binding protein GGA3 isoform X1 [KO:K12404]
108537903  ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 isoform X1 [KO:K12305] [EC:3.6.1.6]
108538130  AP4M1; AP-4 complex subunit mu-1 isoform X1 [KO:K12402]
108538332  NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
108538433  FUCA1; tissue alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
108538647  CTSH; pro-cathepsin H isoform X1 [KO:K01366] [EC:3.4.22.16]
108538848  GGA2; LOW QUALITY PROTEIN: ADP-ribosylation factor-binding protein GGA2 [KO:K12404]
108539152  CTSC; dipeptidyl peptidase 1 [KO:K01275] [EC:3.4.14.1]
108539608  MAN2B1; lysosomal alpha-mannosidase isoform X1 [KO:K12311] [EC:3.2.1.24]
108539780  IDS; iduronate 2-sulfatase isoform X1 [KO:K01136] [EC:3.1.6.13]
108539975  AP1M2; AP-1 complex subunit mu-2 [KO:K12393]
108540011  AP3S1; AP-3 complex subunit sigma-1 isoform X1 [KO:K12399]
108540105  [KO:K12394]
108540639  ATP6V0A2; V-type proton ATPase 116 kDa subunit a [KO:K02154]
108540720  AP3M2; AP-3 complex subunit mu-2 isoform X1 [KO:K12398]
108540839  ABCB9; ATP-binding cassette sub-family B member 9 isoform X1 [KO:K05656]
108541043  WDR7; WD repeat-containing protein 7 [KO:K24738]
108541144  HEXB; LOW QUALITY PROTEIN: beta-hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
108541225  LITAF; lipopolysaccharide-induced tumor necrosis factor-alpha factor [KO:K19363]
108541622  CTSZ; cathepsin Z [KO:K08568] [EC:3.4.18.1]
108542567  CLTA; clathrin light chain A isoform X1 [KO:K04644]
108542762  GLB1; LOW QUALITY PROTEIN: beta-galactosidase [KO:K12309] [EC:3.2.1.23]
108542908  [KO:K05642]
108543015  LAPTM4A; lysosomal-associated transmembrane protein 4A [KO:K12387]
108543135  GNPTAB; N-acetylglucosamine-1-phosphotransferase subunits alpha/beta [KO:K08239] [EC:2.7.8.17]
108543236  TPP1; tripeptidyl-peptidase 1 [KO:K01279] [EC:3.4.14.9]
108543246  SMPD1; sphingomyelin phosphodiesterase [KO:K12350] [EC:3.1.4.12]
108543274  AP1M1; AP-1 complex subunit mu-1 isoform X1 [KO:K12393]
108543421  [KO:K01365] [EC:3.4.22.15]
108543578  NAGA; alpha-N-acetylgalactosaminidase [KO:K01204] [EC:3.2.1.49]
108543676  M6PR; cation-dependent mannose-6-phosphate receptor [KO:K10089]
108543994  AP3B2; AP-3 complex subunit beta-2 [KO:K12397]
108544114  DNASE2; deoxyribonuclease-2-alpha [KO:K01158] [EC:3.1.22.1]
108544384  MFSD8; LOW QUALITY PROTEIN: major facilitator superfamily domain-containing protein 8 [KO:K12307]
108544428  AP3B1; AP-3 complex subunit beta-1 [KO:K12397]
108544584  HEXA; beta-hexosaminidase subunit alpha isoform X1 [KO:K12373] [EC:3.2.1.52]
108544671  AP4S1; AP-4 complex subunit sigma-1 isoform X1 [KO:K12403]
108544804  CLTB; clathrin light chain B isoform X1 [KO:K04645]
108544823  DMXL2; LOW QUALITY PROTEIN: dmX-like protein 2 [KO:K24155]
108544835  AP4E1; AP-4 complex subunit epsilon-1 [KO:K12400]
108545022  ARSA; arylsulfatase A isoform X1 [KO:K01134] [EC:3.1.6.8]
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 22:339-352 (2022)
DOI:10.1038/s41577-021-00626-8
Related
pathway
rbb00511  Other glycan degradation
rbb00531  Glycosaminoglycan degradation
rbb04130  SNARE interactions in vesicular transport
rbb04140  Autophagy - animal
rbb04144  Endocytosis
KO pathway
ko04142   

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