KEGG   PATHWAY: epz04142
Entry
epz04142                    Pathway                                
Name
Lysosome - Equus przewalskii (Przewalski's horse)
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
epz04142  Lysosome
epz04142

Other DBs
GO: 0005764
Organism
Equus przewalskii (Przewalski's horse) [GN:epz]
Gene
103540044  IDS; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
103540431  MCOLN1; mucolipin-1 isoform X1 [KO:K04992]
103540526  ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
103540639  DNASE2; deoxyribonuclease-2-alpha [KO:K01158] [EC:3.1.22.1]
103540706  FUCA1; tissue alpha-L-fucosidase isoform X1 [KO:K01206] [EC:3.2.1.51]
103540950  HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase isoform X3 [KO:K10532] [EC:2.3.1.78]
103541184  AP1S1; AP-1 complex subunit sigma-1A [KO:K12394]
103541283  LAPTM4B; lysosomal-associated transmembrane protein 4B [KO:K12387]
103541297  MANBA; beta-mannosidase isoform X1 [KO:K01192] [EC:3.2.1.25]
103541849  HEXB; beta-hexosaminidase subunit beta isoform X1 [KO:K12373] [EC:3.2.1.52]
103541880  GALNS; N-acetylgalactosamine-6-sulfatase isoform X1 [KO:K01132] [EC:3.1.6.4]
103541990  NAGA; alpha-N-acetylgalactosaminidase [KO:K01204] [EC:3.2.1.49]
103542101  MAN2B1; lysosomal alpha-mannosidase isoform X1 [KO:K12311] [EC:3.2.1.24]
103542159  NAPSA; napsin-A [KO:K08565] [EC:3.4.23.-]
103542275  CTSD; cathepsin D [KO:K01379] [EC:3.4.23.5]
103542463  ATP6V0D1; V-type proton ATPase subunit d 1 isoform X1 [KO:K02146]
103542483  M6PR; cation-dependent mannose-6-phosphate receptor isoform X1 [KO:K10089]
103542499  AP3D1; AP-3 complex subunit delta-1 isoform X1 [KO:K12396]
103542795  GLA; alpha-galactosidase A isoform X1 [KO:K01189] [EC:3.2.1.22]
103543123  SLC17A5; sialin isoform X1 [KO:K12301]
103543192  GGA1; ADP-ribosylation factor-binding protein GGA1 isoform X1 [KO:K12404]
103543754  AP1S2; AP-1 complex subunit sigma-2 isoform X1 [KO:K12394]
103544129  ATP6AP1; V-type proton ATPase subunit S1 isoform X1 [KO:K03662]
103544136  AGA; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase [KO:K01444] [EC:3.5.1.26]
103544212  SPAM1; hyaluronidase PH-20 isoform X1 [KO:K01197] [EC:3.2.1.35]
103545846  SLC11A2; natural resistance-associated macrophage protein 2 isoform X4 [KO:K21398]
103546245  AP3B2; AP-3 complex subunit beta-2 isoform X1 [KO:K12397]
103546574  GGA3; ADP-ribosylation factor-binding protein GGA3 isoform X1 [KO:K12404]
103546923  DMXL2; dmX-like protein 2 isoform X1 [KO:K24155]
103546928  AP4E1; AP-4 complex subunit epsilon-1 isoform X1 [KO:K12400]
103547325  SGSH; N-sulphoglucosamine sulphohydrolase isoform X1 [KO:K01565] [EC:3.10.1.1]
103547327  GAA; lysosomal alpha-glucosidase [KO:K12316] [EC:3.2.1.20]
103547636  CTSS; cathepsin S [KO:K01368] [EC:3.4.22.27]
103547638  CTSK; cathepsin K isoform X1 [KO:K01371] [EC:3.4.22.38]
103547853  CTSC; dipeptidyl peptidase 1 isoform X1 [KO:K01275] [EC:3.4.14.1]
103548098  AP1B1; AP-1 complex subunit beta-1 isoform X1 [KO:K12392]
103548279  ATP6V0B; V-type proton ATPase 21 kDa proteolipid subunit c'' [KO:K03661]
103548318  DMXL1; dmX-like protein 1 isoform X3 [KO:K24155]
103548599  NPC2; NPC intracellular cholesterol transporter 2 [KO:K13443]
103548660  AP3B1; AP-3 complex subunit beta-1 isoform X1 [KO:K12397]
103548666  ARSB; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
103549097  GM2A; ganglioside GM2 activator isoform X1 [KO:K12383]
103549406  PSAP; prosaposin [KO:K12382]
103549471  AP3M1; AP-3 complex subunit mu-1 isoform X1 [KO:K12398]
103549667  LAMP1; lysosome-associated membrane glycoprotein 1 isoform X1 [KO:K06528]
103549713  PLA2G15; lysosomal phospholipase A and acyltransferase isoform X1 [KO:K06129] [EC:3.1.1.5]
103549876  ABCB9; ABC-type oligopeptide transporter ABCB9 [KO:K05656]
103549891  ATP6V0A2; V-type proton ATPase 116 kDa subunit a 2 isoform X1 [KO:K02154]
103550290  SUMF1; formylglycine-generating enzyme isoform X1 [KO:K13444] [EC:1.8.3.7]
103550310  AP1S3; AP-1 complex subunit sigma-3 [KO:K12395]
103550363  ATP6V0A1; V-type proton ATPase 116 kDa subunit a 1 isoform X1 [KO:K02154]
103550388  NAGLU; alpha-N-acetylglucosaminidase [KO:K01205] [EC:3.2.1.50]
103550751  ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 isoform X1 [KO:K12305] [EC:3.6.1.6]
103550840  CTSL; procathepsin L isoform X1 [KO:K01365] [EC:3.4.22.15]
103551138  CD164; sialomucin core protein 24 [KO:K06546]
103551282  TCIRG1; V-type proton ATPase 116 kDa subunit a 3 isoform X1 [KO:K02154]
103551640  ATP6V0C; V-type proton ATPase 16 kDa proteolipid subunit c [KO:K02155]
103551682  WDR7; WD repeat-containing protein 7 isoform X1 [KO:K24738]
103551741  AP1G1; AP-1 complex subunit gamma-1 [KO:K12391]
103551800  GALC; galactocerebrosidase isoform X6 [KO:K01202] [EC:3.2.1.46]
103551923  CTSA; lysosomal protective protein [KO:K13289] [EC:3.4.16.5]
103552714  AP4S1; AP-4 complex subunit sigma-1 isoform X1 [KO:K12403]
103552844  GBA1; lysosomal acid glucosylceramidase [KO:K01201] [EC:3.2.1.45]
103553061  SORT1; sortilin isoform X3 [KO:K12388]
103553536  TPP1; tripeptidyl-peptidase 1 isoform X1 [KO:K01279] [EC:3.4.14.9]
103553563  PPT1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
103553838  CTSE; cathepsin E [KO:K01382] [EC:3.4.23.34]
103553896  PSAPL1; proactivator polypeptide-like 1 [KO:K12382]
103554014  MEAK7; MTOR-associated protein MEAK7 isoform X5 [KO:K27947]
103554778  CLN5; bis(monoacylglycero)phosphate synthase CLN5 isoform X1 [KO:K12390]
103555469  HEXA; beta-hexosaminidase subunit alpha isoform X1 [KO:K12373] [EC:3.2.1.52]
103555699  GNPTAB; N-acetylglucosamine-1-phosphotransferase subunits alpha/beta isoform X1 [KO:K08239] [EC:2.7.8.17]
103556309  ASAH1; acid ceramidase isoform X1 [KO:K12348] [EC:3.5.1.23]
103556436  LAPTM4A; lysosomal-associated transmembrane protein 4A [KO:K12387]
103556526  LGMN; legumain [KO:K01369] [EC:3.4.22.34]
103557145  LITAF; lipopolysaccharide-induced tumor necrosis factor-alpha factor isoform X1 [KO:K19363]
103557287  DNASE2B; deoxyribonuclease-2-beta isoform X1 [KO:K01158] [EC:3.1.22.1]
103557348  HYAL3; hyaluronidase-3 isoform X1 [KO:K01197] [EC:3.2.1.35]
103557349  HYAL2; hyaluronidase-2 isoform X1 [KO:K01197] [EC:3.2.1.35]
103557712  CD68; macrosialin [KO:K06501]
103557809  GNS; N-acetylglucosamine-6-sulfatase isoform X1 [KO:K01137] [EC:3.1.6.14]
103558035  GUSB; beta-glucuronidase isoform X1 [KO:K01195] [EC:3.2.1.31]
103558180  CTSB; cathepsin B [KO:K01363] [EC:3.4.22.1]
103558193  LAMP3; lysosome-associated membrane glycoprotein 3 isoform X1 [KO:K06562]
103558370  AP1M1; AP-1 complex subunit mu-1 isoform X1 [KO:K12393]
103558448  IGF2R; cation-independent mannose-6-phosphate receptor isoform X1 [KO:K06564]
103558473  ARSG; arylsulfatase G isoform X1 [KO:K12381] [EC:3.1.6.-]
103558522  FUCA2; plasma alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
103559011  CTSH; pro-cathepsin H [KO:K01366] [EC:3.4.22.16]
103559113  GNPTG; N-acetylglucosamine-1-phosphotransferase subunit gamma isoform X1 [KO:K10087]
103559330  ATP6V0A4; V-type proton ATPase 116 kDa subunit a 4 [KO:K02154]
103559688  CTSF; cathepsin F [KO:K01373] [EC:3.4.22.41]
103560080  CTSW; cathepsin W isoform X1 [KO:K08569] [EC:3.4.22.-]
103560205  CD63; CD63 antigen [KO:K06497]
103560370  LAPTM5; lysosomal-associated transmembrane protein 5 [KO:K12387]
103560648  [KO:K01197] [EC:3.2.1.35]
103561062  [KO:K01319] [EC:3.4.21.20]
103561107  GLB1; beta-galactosidase isoform X1 [KO:K12309] [EC:3.2.1.23]
103561501  NPC1; NPC intracellular cholesterol transporter 1 isoform X2 [KO:K12385]
103561773  AP4M1; AP-4 complex subunit mu-1 isoform X1 [KO:K12402]
103562214  SCARB2; lysosome membrane protein 2 isoform X1 [KO:K12384]
103562319  ACP5; tartrate-resistant acid phosphatase type 5 [KO:K14379] [EC:3.1.3.2]
103562419  AP1M2; AP-1 complex subunit mu-2 isoform X1 [KO:K12393]
103562665  GGA2; ADP-ribosylation factor-binding protein GGA2 isoform X1 [KO:K12404]
103562739  CLN3; battenin isoform X1 [KO:K12389]
103562824  MFSD8; major facilitator superfamily domain-containing protein 8 isoform X1 [KO:K12307]
103562904  AP3M2; AP-3 complex subunit mu-2 isoform X1 [KO:K12398]
103562930  CTNS; cystinosin isoform X1 [KO:K12386]
103563007  AP1G2; AP-1 complex subunit gamma-like 2 isoform X1 [KO:K12391]
103563128  NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase isoform X1 [KO:K01125] [EC:3.1.4.45]
103563220  [KO:K01365] [EC:3.4.22.15]
103563289  NEU1; sialidase-1 [KO:K01186] [EC:3.2.1.18]
103563364  CLTB; clathrin light chain B isoform X1 [KO:K04645]
103564154  SLC11A1; natural resistance-associated macrophage protein 1 [KO:K12347]
103564255  LAMP2; lysosome-associated membrane glycoprotein 2 isoform X1 [KO:K06528]
103564405  CLTA; clathrin light chain A isoform X1 [KO:K04644]
103565375  IDUA; alpha-L-iduronidase isoform X1 [KO:K01217] [EC:3.2.1.76]
103565379  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
103565404  CTSZ; cathepsin Z [KO:K08568] [EC:3.4.18.1]
103565932  AP4B1; AP-4 complex subunit beta-1 [KO:K12401]
103566588  LIPA; lysosomal acid lipase/cholesteryl ester hydrolase isoform X1 [KO:K01052] [EC:3.1.1.13]
103566642  PPT2; lysosomal thioesterase PPT2 isoform X1 [KO:K01074] [EC:3.1.2.22]
103566744  ACP2; lysosomal acid phosphatase [KO:K14410] [EC:3.1.3.2]
103566812  CTSO; cathepsin O [KO:K01374] [EC:3.4.22.42]
103567788  ATP6V1H; V-type proton ATPase subunit H isoform X1 [KO:K02144]
103567953  ATP6V0D2; V-type proton ATPase subunit d 2 isoform X1 [KO:K02146]
103568007  NCOA7; nuclear receptor coactivator 7 isoform X1 [KO:K25442]
103568012  ABCA2; ATP-binding cassette sub-family A member 2 [KO:K05642]
139082900  [KO:K01197] [EC:3.2.1.35]
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
epz00511  Other glycan degradation
epz00531  Glycosaminoglycan degradation
epz04130  SNARE interactions in vesicular transport
epz04140  Autophagy - animal
epz04144  Endocytosis
KO pathway
ko04142   

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