KEGG   PATHWAY: pon04142
Entry
pon04142                    Pathway                                
Name
Lysosome - Pongo abelii (Sumatran orangutan)
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
pon04142  Lysosome
pon04142

Other DBs
GO: 0005764
Organism
Pongo abelii (Sumatran orangutan) [GN:pon]
Gene
100173661  ATP6V0A1; V-type proton ATPase 116 kDa subunit a isoform 1 [KO:K02154]
100451994  TCIRG1; V-type proton ATPase 116 kDa subunit a [KO:K02154]
100939293  ATP6V0A2; V-type proton ATPase 116 kDa subunit a isoform X1 [KO:K02154]
100460887  ATP6V0A4; V-type proton ATPase 116 kDa subunit a [KO:K02154]
100173531  ATP6V0D1; V-type proton ATPase subunit d 1 [KO:K02146]
100173359  ATP6V0D2; V-type proton ATPase subunit d 2 [KO:K02146]
100173044  ATP6V1H; V-type proton ATPase subunit H [KO:K02144]
100173990  ATP6AP1; V-type proton ATPase subunit S1 [KO:K03662]
100452031  ATP6V0C; V-type proton ATPase 16 kDa proteolipid subunit [KO:K02155]
100440396  ATP6V0B; V-type proton ATPase 21 kDa proteolipid subunit isoform X1 [KO:K03661]
100448171  DMXL1; dmX-like protein 1 isoform X1 [KO:K24155]
100440761  DMXL2; dmX-like protein 2 [KO:K24155]
100446424  WDR7; WD repeat-containing protein 7 [KO:K24738]
100448771  NCOA7; nuclear receptor coactivator 7 isoform X2 [KO:K25442]
100173381  CTSA; lysosomal protective protein precursor [KO:K13289] [EC:3.4.16.5]
100173564  CTSB; cathepsin B precursor [KO:K01363] [EC:3.4.22.1]
100172530  CTSC; dipeptidyl peptidase 1 precursor [KO:K01275] [EC:3.4.14.1]
100172401  CTSD; cathepsin D precursor [KO:K01379] [EC:3.4.23.5]
100452388  CTSE; cathepsin E isoform X1 [KO:K01382] [EC:3.4.23.34]
100439389  CTSF; LOW QUALITY PROTEIN: cathepsin F [KO:K01373] [EC:3.4.22.41]
100436066  CTSG; cathepsin G [KO:K01319] [EC:3.4.21.20]
100460934  CTSH; pro-cathepsin H isoform X1 [KO:K01366] [EC:3.4.22.16]
100432447  CTSK; cathepsin K [KO:K01371] [EC:3.4.22.38]
100450374  CTSL; cathepsin L1 [KO:K01365] [EC:3.4.22.15]
100452609  putative inactive cathepsin L-like protein CTSL3P [KO:K01365] [EC:3.4.22.15]
100440226  LOW QUALITY PROTEIN: cathepsin L1-like [KO:K01365] [EC:3.4.22.15]
100435624  CTSO; cathepsin O [KO:K01374] [EC:3.4.22.42]
100432810  CTSS; cathepsin S isoform X1 [KO:K01368] [EC:3.4.22.27]
100439744  CTSV; cathepsin L2 isoform X2 [KO:K01375] [EC:3.4.22.43]
100453245  CTSW; cathepsin W [KO:K08569] [EC:3.4.22.-]
100445547  CTSZ; cathepsin Z isoform X1 [KO:K08568] [EC:3.4.18.1]
100455134  napsin-A [KO:K08565] [EC:3.4.23.-]
100455861  napsin-A [KO:K08565] [EC:3.4.23.-]
100173793  LGMN; legumain precursor [KO:K01369] [EC:3.4.22.34]
100440001  TPP1; tripeptidyl-peptidase 1 precursor [KO:K01279] [EC:3.4.14.9]
100444354  GLA; alpha-galactosidase A [KO:K01189] [EC:3.2.1.22]
100173272  GLB1; beta-galactosidase precursor [KO:K12309] [EC:3.2.1.23]
100173365  GAA; lysosomal alpha-glucosidase precursor [KO:K12316] [EC:3.2.1.20]
100174563  GBA; glucosylceramidase precursor [KO:K01201] [EC:3.2.1.45]
112128502  LOW QUALITY PROTEIN: glucosylceramidase-like [KO:K01201] [EC:3.2.1.45]
100458752  IDUA; alpha-L-iduronidase isoform X1 [KO:K01217] [EC:3.2.1.76]
100450338  NAGA; alpha-N-acetylgalactosaminidase isoform X1 [KO:K01204] [EC:3.2.1.49]
100455729  NAGLU; alpha-N-acetylglucosaminidase [KO:K01205] [EC:3.2.1.50]
100458475  GALC; galactocerebrosidase [KO:K01202] [EC:3.2.1.46]
100173726  GUSB; beta-glucuronidase precursor [KO:K01195] [EC:3.2.1.31]
100439268  beta-glucuronidase-like isoform X1 [KO:K01195] [EC:3.2.1.31]
100174203  FUCA2; plasma alpha-L-fucosidase precursor [KO:K01206] [EC:3.2.1.51]
100452896  FUCA1; tissue alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
100438794  HEXA; beta-hexosaminidase subunit alpha precursor [KO:K12373] [EC:3.2.1.52]
100438599  HEXB; beta-hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
100171985  MANBA; beta-mannosidase precursor [KO:K01192] [EC:3.2.1.25]
100174476  MAN2B1; lysosomal alpha-mannosidase precursor [KO:K12311] [EC:3.2.1.24]
100172668  NEU1; sialidase-1 isoform 1 precursor [KO:K01186] [EC:3.2.1.18]
100458070  hyaluronidase-4 [KO:K01197] [EC:3.2.1.35]
100462117  SPAM1; hyaluronidase PH-20 isoform X1 [KO:K01197] [EC:3.2.1.35]
100453650  HYAL2; hyaluronidase-2 [KO:K01197] [EC:3.2.1.35]
100455010  HYAL1; hyaluronidase-1 isoform X1 [KO:K01197] [EC:3.2.1.35]
100455370  HYAL3; hyaluronidase-3 [KO:K01197] [EC:3.2.1.35]
100461750  LOW QUALITY PROTEIN: hyaluronidase-4-like [KO:K01197] [EC:3.2.1.35]
100446820  ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
100457196  ARSB; arylsulfatase B isoform X1 [KO:K01135] [EC:3.1.6.12]
100447099  ARSG; arylsulfatase G isoform X1 [KO:K12381] [EC:3.1.6.-]
100457027  GALNS; N-acetylgalactosamine-6-sulfatase isoform X1 [KO:K01132] [EC:3.1.6.4]
100434320  GNS; N-acetylglucosamine-6-sulfatase [KO:K01137] [EC:3.1.6.14]
100435640  IDS; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
100459478  SGSH; N-sulphoglucosamine sulphohydrolase isoform X1 [KO:K01565] [EC:3.10.1.1]
100453604  LIPA; lysosomal acid lipase/cholesteryl ester hydrolase [KO:K01052] [EC:3.1.1.13]
100172651  PLA2G15; group XV phospholipase A2 precursor [KO:K06129] [EC:3.1.1.5]
100171654  DNASE2; deoxyribonuclease-2-alpha precursor [KO:K01158] [EC:3.1.22.1]
100453264  DNASE2B; deoxyribonuclease-2-beta [KO:K01158] [EC:3.1.22.1]
100174154  ACP2; lysosomal acid phosphatase precursor [KO:K14410] [EC:3.1.3.2]
100458870  ACP5; tartrate-resistant acid phosphatase type 5 [KO:K14379] [EC:3.1.3.2]
100172518  SMPD1; sphingomyelin phosphodiesterase [KO:K12350] [EC:3.1.4.12]
100171626  ASAH1; acid ceramidase precursor [KO:K12348] [EC:3.5.1.23]
100453921  AGA; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase [KO:K01444] [EC:3.5.1.26]
100174080  PSAP; prosaposin isoform 2 [KO:K12382]
100939793  PSAPL1; proactivator polypeptide-like 1 [KO:K12382]
100441787  GM2A; ganglioside GM2 activator isoform X1 [KO:K12383]
100442697  LOW QUALITY PROTEIN: ganglioside GM2 activator-like [KO:K12383]
100434271  PPT2; lysosomal thioesterase PPT2 isoform X1 [KO:K01074] [EC:3.1.2.22]
100174770  PPT1; palmitoyl-protein thioesterase 1 precursor [KO:K01074] [EC:3.1.2.22]
100456181  LAMP1; lysosome-associated membrane glycoprotein 1 [KO:K06528]
100438981  LAMP2; lysosome-associated membrane glycoprotein 2 isoform X2 [KO:K06528]
100437850  LAMP3; lysosome-associated membrane glycoprotein 3 [KO:K06562]
100432829  CD68; macrosialin [KO:K06501]
100438311  CD63; CD63 antigen [KO:K06497]
100455864  SCARB2; lysosome membrane protein 2 [KO:K12384]
100441015  NPC1; Niemann-Pick C1 protein [KO:K12385]
100457753  NPC2; epididymal secretory protein E1 isoform X1 [KO:K13443]
100443212  CTNS; cystinosin isoform X3 [KO:K12386]
100460012  SLC17A5; sialin isoform X1 [KO:K12301]
100446791  SLC11A1; natural resistance-associated macrophage protein 1 isoform X1 [KO:K12347]
100434194  SLC11A2; natural resistance-associated macrophage protein 2 isoform X1 [KO:K21398]
100174459  LAPTM4A; lysosomal-associated transmembrane protein 4A [KO:K12387]
100171603  LAPTM5; lysosomal-associated transmembrane protein 5 [KO:K12387]
100445214  LAPTM4B; LOW QUALITY PROTEIN: lysosomal-associated transmembrane protein 4B [KO:K12387]
100454242  lysosomal-associated transmembrane protein 4B-like [KO:K12387]
100461885  ABCA2; ATP-binding cassette sub-family A member 2 isoform X1 [KO:K05642]
100444287  ABCB9; ATP-binding cassette sub-family B member 9 isoform X1 [KO:K05656]
100454546  CD164; sialomucin core protein 24 isoform X1 [KO:K06546]
100433776  ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 isoform X1 [KO:K12305] [EC:3.6.1.6]
100457993  SORT1; sortilin isoform X1 [KO:K12388]
100457403  CLN3; battenin isoform X2 [KO:K12389]
100454233  CLN5; ceroid-lipofuscinosis neuronal protein 5 [KO:K12390]
100434258  MFSD8; major facilitator superfamily domain-containing protein 8 isoform X1 [KO:K12307]
100447325  HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase isoform X1 [KO:K10532] [EC:2.3.1.78]
100446797  SUMF1; sulfatase-modifying factor 1 isoform X1 [KO:K13444] [EC:1.8.3.7]
100441852  GNPTAB; N-acetylglucosamine-1-phosphotransferase subunits alpha/beta isoform X1 [KO:K08239] [EC:2.7.8.17]
100455711  GNPTG; N-acetylglucosamine-1-phosphotransferase subunit gamma isoform X1 [KO:K10087]
100458605  NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
100432010  IGF2R; cation-independent mannose-6-phosphate receptor [KO:K06564]
100443312  M6PR; cation-dependent mannose-6-phosphate receptor [KO:K10089]
100461265  CLTA; clathrin light chain A [KO:K04644]
100454415  CLTB; clathrin light chain B [KO:K04645]
100434550  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
100450082  CLTCL1; clathrin heavy chain 2 [KO:K04646]
100441449  AP1G2; AP-1 complex subunit gamma-like 2 isoform X2 [KO:K12391]
100173736  AP1G1; AP-1 complex subunit gamma-1 [KO:K12391]
100436759  AP1B1; AP-1 complex subunit beta-1 isoform X1 [KO:K12392]
100441763  AP1M1; AP-1 complex subunit mu-1 [KO:K12393]
100442364  AP1M2; AP-1 complex subunit mu-2 isoform X1 [KO:K12393]
100437759  AP1S1; AP-1 complex subunit sigma-1A [KO:K12394]
100435994  AP1S2; AP-1 complex subunit sigma-2 isoform X2 [KO:K12394]
100435961  AP1S3; AP-1 complex subunit sigma-3 isoform X1 [KO:K12395]
100450198  AP3D1; LOW QUALITY PROTEIN: AP-3 complex subunit delta-1 [KO:K12396]
100456119  AP3B1; AP-3 complex subunit beta-1 isoform X1 [KO:K12397]
100453347  AP3B2; AP-3 complex subunit beta-2 isoform X1 [KO:K12397]
100439972  AP3M2; AP-3 complex subunit mu-2 isoform X1 [KO:K12398]
100174037  AP3M1; AP-3 complex subunit mu-1 [KO:K12398]
100171891  AP3S2; AP-3 complex subunit sigma-2 [KO:K12399]
100440806  AP3S1; AP-3 complex subunit sigma-1 [KO:K12399]
100438792  AP4E1; AP-4 complex subunit epsilon-1 isoform X1 [KO:K12400]
100447996  AP4B1; AP-4 complex subunit beta-1 isoform X1 [KO:K12401]
100174275  AP4M1; AP-4 complex subunit mu-1 [KO:K12402]
100443568  AP4S1; AP-4 complex subunit sigma-1 isoform X1 [KO:K12403]
100172741  GGA3; ADP-ribosylation factor-binding protein GGA3 [KO:K12404]
100437381  GGA1; ADP-ribosylation factor-binding protein GGA1 isoform X1 [KO:K12404]
100455667  uncharacterized protein LOC100455667 [KO:K12404]
100453649  MCOLN1; mucolipin-1 isoform X1 [KO:K04992]
100447960  LITAF; LOW QUALITY PROTEIN: lipopolysaccharide-induced tumor necrosis factor-alpha factor [KO:K19363]
112131934  lipopolysaccharide-induced tumor necrosis factor-alpha factor-like [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
pon00511  Other glycan degradation
pon00531  Glycosaminoglycan degradation
pon04130  SNARE interactions in vesicular transport
pon04140  Autophagy - animal
pon04144  Endocytosis
KO pathway
ko04142   
LinkDB

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