KEGG   PATHWAY: oas04142
Entry
oas04142                    Pathway                                
Name
Lysosome - Ovis aries (sheep)
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
oas04142  Lysosome
oas04142

Other DBs
GO: 0005764
Organism
Ovis aries (sheep) [GN:oas]
Gene
100034668  CLN5; ceroid-lipofuscinosis neuronal protein 5 precursor [KO:K12390]
100037669  NPC2; NPC intracellular cholesterol transporter 2 [KO:K13443]
100135695  PSAP; prosaposin isoform X1 [KO:K12382]
100145856  HEXA; beta-hexosaminidase subunit alpha precursor [KO:K12373] [EC:3.2.1.52]
100145858  NAGA; alpha-N-acetylgalactosaminidase precursor [KO:K01204] [EC:3.2.1.49]
100145859  LIPA; lysosomal acid lipase/cholesteryl ester hydrolase precursor [KO:K01052] [EC:3.1.1.13]
100307047  SMPD1; sphingomyelin phosphodiesterase [KO:K12350] [EC:3.1.4.12]
100820744  ACP5; tartrate-resistant acid phosphatase type 5 precursor [KO:K14379] [EC:3.1.3.2]
101101889  [KO:K01197] [EC:3.2.1.35]
101101911  CTSZ; cathepsin Z [KO:K08568] [EC:3.4.18.1]
101102037  HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase isoform X2 [KO:K10532] [EC:2.3.1.78]
101102143  [KO:K01197] [EC:3.2.1.35]
101102146  MAN2B1; lysosomal alpha-mannosidase [KO:K12311] [EC:3.2.1.24]
101102252  NAGLU; alpha-N-acetylglucosaminidase [KO:K01205] [EC:3.2.1.50]
101102500  CD68; macrosialin isoform X1 [KO:K06501]
101102802  M6PR; cation-dependent mannose-6-phosphate receptor isoform X1 [KO:K10089]
101103171  MEAK7; MTOR-associated protein MEAK7 isoform X2 [KO:K27947]
101103444  LAMP3; lysosome-associated membrane glycoprotein 3 isoform X1 [KO:K06562]
101103549  LAPTM4A; lysosomal-associated transmembrane protein 4A [KO:K12387]
101104002  AP1G1; AP-1 complex subunit gamma-1 isoform X1 [KO:K12391]
101104057  ATP6V0A4; V-type proton ATPase 116 kDa subunit a 4 isoform X1 [KO:K02154]
101104538  SORT1; sortilin isoform X7 [KO:K12388]
101104545  NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
101104633  AP1S2; AP-1 complex subunit sigma-2 isoform X1 [KO:K12394]
101105079  DMXL2; dmX-like protein 2 isoform X2 [KO:K24155]
101105107  AP3S2; AP-3 complex subunit sigma-2 [KO:K12399]
101105138  [KO:K06497]
101105493  DNASE2; deoxyribonuclease-2-alpha [KO:K01158] [EC:3.1.22.1]
101105496  GM2A; ganglioside GM2 activator [KO:K12383]
101105502  SCARB2; lysosome membrane protein 2 isoform X2 [KO:K12384]
101105605  AP3B2; AP-3 complex subunit beta-2 isoform X2 [KO:K12397]
101105916  IDUA; alpha-L-iduronidase isoform X1 [KO:K01217] [EC:3.2.1.76]
101106174  AP4E1; AP-4 complex subunit epsilon-1 [KO:K12400]
101106236  GLA; alpha-galactosidase A [KO:K01189] [EC:3.2.1.22]
101106396  AGA; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase [KO:K01444] [EC:3.5.1.26]
101106519  HYAL4; hyaluronidase-4 isoform X1 [KO:K01197] [EC:3.2.1.35]
101106572  CLTA; clathrin light chain A isoform X1 [KO:K04644]
101107107  LAPTM4B; lysosomal-associated transmembrane protein 4B isoform X1 [KO:K12387]
101107114  CLTC; clathrin heavy chain 1 isoform X2 [KO:K04646]
101107188  [KO:K01197] [EC:3.2.1.35]
101107214  GALNS; N-acetylgalactosamine-6-sulfatase isoform X1 [KO:K01132] [EC:3.1.6.4]
101107603  AP3S1; AP-3 complex subunit sigma-1 isoform X1 [KO:K12399]
101107757  AP4M1; AP-4 complex subunit mu-1 [KO:K12402]
101107963  MANBA; beta-mannosidase [KO:K01192] [EC:3.2.1.25]
101108314  ARSB; arylsulfatase B isoform X1 [KO:K01135] [EC:3.1.6.12]
101108427  CTSH; pro-cathepsin H [KO:K01366] [EC:3.4.22.16]
101108446  GNPTG; N-acetylglucosamine-1-phosphotransferase subunit gamma [KO:K10087]
101108509  ATP6V0D1; V-type proton ATPase subunit d 1 isoform X2 [KO:K02146]
101108802  GUSB; beta-glucuronidase [KO:K01195] [EC:3.2.1.31]
101108972  CTSK; cathepsin K isoform X1 [KO:K01371] [EC:3.4.22.38]
101109286  ARSG; arylsulfatase G isoform X1 [KO:K12381] [EC:3.1.6.-]
101109408  LITAF; lipopolysaccharide-induced tumor necrosis factor-alpha factor isoform X2 [KO:K19363]
101110003  SUMF1; formylglycine-generating enzyme isoform X1 [KO:K13444] [EC:1.8.3.7]
101110100  DNASE2B; deoxyribonuclease-2-beta [KO:K01158] [EC:3.1.22.1]
101110316  GNS; N-acetylglucosamine-6-sulfatase [KO:K01137] [EC:3.1.6.14]
101110428  GGA3; ADP-ribosylation factor-binding protein GGA3 isoform X1 [KO:K12404]
101110464  CTSW; cathepsin W [KO:K08569] [EC:3.4.22.-]
101111169  ABCA2; ATP-binding cassette sub-family A member 2 isoform X4 [KO:K05642]
101111906  HEXB; beta-hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
101112162  [KO:K12373] [EC:3.2.1.52]
101112650  AP3B1; AP-3 complex subunit beta-1 [KO:K12397]
101112686  CTSC; dipeptidyl peptidase 1 isoform X1 [KO:K01275] [EC:3.4.14.1]
101112852  CTSO; cathepsin O isoform X1 [KO:K01374] [EC:3.4.22.42]
101112855  PPT2; lysosomal thioesterase PPT2 isoform X3 [KO:K01074] [EC:3.1.2.22]
101112930  GLB1; beta-galactosidase [KO:K12309] [EC:3.2.1.23]
101112942  CLN3; battenin isoform X2 [KO:K12389]
101113184  TCIRG1; V-type proton ATPase 116 kDa subunit a 3 [KO:K02154]
101113307  LAMP2; lysosome-associated membrane glycoprotein 2 isoform X3 [KO:K06528]
101113504  ATP6V1H; V-type proton ATPase subunit H isoform X2 [KO:K02144]
101113615  CTSA; lysosomal protective protein isoform X2 [KO:K13289] [EC:3.4.16.5]
101113725  CTSL; procathepsin L [KO:K01365] [EC:3.4.22.15]
101114221  ATP6V0B; V-type proton ATPase 21 kDa proteolipid subunit c'' isoform X2 [KO:K03661]
101114351  AP1M2; AP-1 complex subunit mu-2 isoform X3 [KO:K12393]
101114873  CD164; sialomucin core protein 24 isoform X1 [KO:K06546]
101114944  CD63; CD63 antigen [KO:K06497]
101115363  DMXL1; dmX-like protein 1 isoform X9 [KO:K24155]
101115405  AP4S1; AP-4 complex subunit sigma-1 [KO:K12403]
101115614  SLC11A2; natural resistance-associated macrophage protein 2 isoform X1 [KO:K21398]
101116140  AP1M1; AP-1 complex subunit mu-1 [KO:K12393]
101116391  [KO:K21398]
101116435  NEU1; sialidase-1 [KO:K01186] [EC:3.2.1.18]
101116503  NAPSA; napsin-A [KO:K08565] [EC:3.4.23.-]
101116540  IDS; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
101116858  TPP1; tripeptidyl-peptidase 1 [KO:K01279] [EC:3.4.14.9]
101116975  [KO:K21398]
101117016  ACP2; lysosomal acid phosphatase isoform X1 [KO:K14410] [EC:3.1.3.2]
101117133  GGA2; ADP-ribosylation factor-binding protein GGA2 [KO:K12404]
101117145  AP1S3; AP-1 complex subunit sigma-3 isoform X4 [KO:K12395]
101117328  CLTB; clathrin light chain B isoform X2 [KO:K04645]
101117379  GBA1; lysosomal acid glucosylceramidase isoform X1 [KO:K01201] [EC:3.2.1.45]
101117677  ATP6V0D2; V-type proton ATPase subunit d 2 [KO:K02146]
101117768  NCOA7; nuclear receptor coactivator 7 isoform X2 [KO:K25442]
101117904  ASAH1; acid ceramidase isoform X1 [KO:K12348] [EC:3.5.1.23]
101118077  AP3M2; AP-3 complex subunit mu-2 [KO:K12398]
101118106  GALC; galactocerebrosidase isoform X4 [KO:K01202] [EC:3.2.1.46]
101118294  PLA2G15; phospholipase A2 group XV isoform X1 [KO:K06129] [EC:3.1.1.5]
101118364  FUCA2; plasma alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
101119252  ABCB9; ABC-type oligopeptide transporter ABCB9 isoform X1 [KO:K05656]
101119310  AP1G2; AP-1 complex subunit gamma-like 2 isoform X1 [KO:K12391]
101119403  LAMP1; lysosome-associated membrane glycoprotein 1 [KO:K06528]
101119449  FUCA1; tissue alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
101119886  ATP6AP1; V-type proton ATPase subunit S1 [KO:K03662]
101120555  ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 isoform X1 [KO:K12305] [EC:3.6.1.6]
101120623  NPC1; NPC intracellular cholesterol transporter 1 isoform X1 [KO:K12385]
101120710  AP4B1; AP-4 complex subunit beta-1 isoform X2 [KO:K12401]
101121049  PPT1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
101121184  SGSH; N-sulphoglucosamine sulphohydrolase isoform X1 [KO:K01565] [EC:3.10.1.1]
101121207  HYAL1; hyaluronidase-1 [KO:K01197] [EC:3.2.1.35]
101121298  [KO:K01319] [EC:3.4.21.20]
101121461  HYAL3; hyaluronidase-3 isoform X1 [KO:K01197] [EC:3.2.1.35]
101121550  ATP6V0A2; V-type proton ATPase 116 kDa subunit a 2 isoform X1 [KO:K02154]
101121553  [KO:K01319] [EC:3.4.21.20]
101121575  LAPTM5; lysosomal-associated transmembrane protein 5 [KO:K12387]
101121692  GAA; lysosomal alpha-glucosidase isoform X2 [KO:K12316] [EC:3.2.1.20]
101121711  MFSD8; major facilitator superfamily domain-containing protein 8 isoform X1 [KO:K12307]
101121713  AP1B1; AP-1 complex subunit beta-1 isoform X1 [KO:K12392]
101121852  AP3D1; AP-3 complex subunit delta-1 [KO:K12396]
101121931  MCOLN1; mucolipin-1 isoform X1 [KO:K04992]
101122069  WDR7; WD repeat-containing protein 7 isoform X2 [KO:K24738]
101122075  AP3M1; AP-3 complex subunit mu-1 [KO:K12398]
101122266  ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
101122348  GNPTAB; N-acetylglucosamine-1-phosphotransferase subunits alpha/beta isoform X1 [KO:K08239] [EC:2.7.8.17]
101122493  AP1S1; AP-1 complex subunit sigma-1A isoform X2 [KO:K12394]
101122533  CTNS; cystinosin [KO:K12386]
101123025  GGA1; ADP-ribosylation factor-binding protein GGA1 isoform X1 [KO:K12404]
101123476  ATP6V0A1; V-type proton ATPase 116 kDa subunit a 1 isoform X3 [KO:K02154]
105602066  [KO:K06497]
105604650  LITAFD; lITAF domain-containing protein [KO:K19363]
105605805  [KO:K01195] [EC:3.2.1.31]
105615572  PSAPL1; proactivator polypeptide-like 1 [KO:K12382]
114110441  [KO:K12385]
114112781  [KO:K12387]
443003  ATP6V0C; V-type proton ATPase 16 kDa proteolipid subunit c [KO:K02155]
443060  CTSD; cathepsin D isoform X2 [KO:K01379] [EC:3.4.23.5]
443098  SLC17A5; sialin [KO:K12301]
443154  HYAL2; hyaluronidase-2 precursor [KO:K01197] [EC:3.2.1.35]
443365  SLC11A1; natural resistance-associated macrophage protein 1 [KO:K12347]
443500  IGF2R; cation-independent mannose-6-phosphate receptor [KO:K06564]
678680  LGMN; legumain isoform X2 [KO:K01369] [EC:3.4.22.34]
780470  CTSB; cathepsin B precursor [KO:K01363] [EC:3.4.22.1]
780471  CTSF; cathepsin F isoform X1 [KO:K01373] [EC:3.4.22.41]
780472  CTSS; cathepsin S isoform X1 [KO:K01368] [EC:3.4.22.27]
780510  [KO:K01365] [EC:3.4.22.15]
Compound
C00002  ATP
C00008  ADP
C00159  D-Mannose
C00275  D-Mannose 6-phosphate
Reference
PMID:12628346
  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
PMID:15987739
  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
PMID:18635948
  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
PMID:34249946
  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
PMID:34646033
  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
oas00511  Other glycan degradation
oas00531  Glycosaminoglycan degradation
oas04130  SNARE interactions in vesicular transport
oas04140  Autophagy - animal
oas04144  Endocytosis
KO pathway
ko04142   

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