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
103549891  ATP6V0A2; ATPase H+ transporting V0 subunit a2 [KO:K02154]
103550363  ATP6V0A1; ATPase H+ transporting V0 subunit a1 [KO:K02154]
103559330  ATP6V0A4; ATPase H+ transporting V0 subunit a4 [KO:K02154]
103542463  ATP6V0D1; ATPase H+ transporting V0 subunit d1 [KO:K02146]
103567953  ATP6V0D2; ATPase H+ transporting V0 subunit d2 [KO:K02146]
103567788  ATP6V1H; ATPase H+ transporting V1 subunit H [KO:K02144]
103544129  ATP6AP1; ATPase H+ transporting accessory protein 1 [KO:K03662]
103551640  ATP6V0C; ATPase H+ transporting V0 subunit c [KO:K02155]
103561720  V-type proton ATPase 16 kDa proteolipid subunit [KO:K02155]
103548279  ATP6V0B; ATPase H+ transporting V0 subunit b [KO:K03661]
103548318  DMXL1; Dmx like 1 [KO:K24155]
103546923  DMXL2; Dmx like 2 [KO:K24155]
103551682  WDR7; WD repeat domain 7 [KO:K24738]
103568007  NCOA7; nuclear receptor coactivator 7 [KO:K25442]
103551923  CTSA; cathepsin A [KO:K13289] [EC:3.4.16.5]
103558180  CTSB; cathepsin B [KO:K01363] [EC:3.4.22.1]
103547853  CTSC; cathepsin C [KO:K01275] [EC:3.4.14.1]
103542275  CTSD; cathepsin D [KO:K01379] [EC:3.4.23.5]
103553838  CTSE; cathepsin E [KO:K01382] [EC:3.4.23.34]
103559688  CTSF; cathepsin F [KO:K01373] [EC:3.4.22.41]
103561062  CTSG; cathepsin G [KO:K01319] [EC:3.4.21.20]
103559011  CTSH; cathepsin H [KO:K01366] [EC:3.4.22.16]
103547638  CTSK; cathepsin K [KO:K01371] [EC:3.4.22.38]
103563220  cathepsin L1 [KO:K01365] [EC:3.4.22.15]
103550840  CTSL; cathepsin L [KO:K01365] [EC:3.4.22.15]
103566812  CTSO; cathepsin O [KO:K01374] [EC:3.4.22.42]
103547636  CTSS; cathepsin S [KO:K01368] [EC:3.4.22.27]
103560080  CTSW; cathepsin W [KO:K08569] [EC:3.4.22.-]
103545980  cathepsin W-like [KO:K08569] [EC:3.4.22.-]
103565404  CTSZ; cathepsin Z [KO:K08568] [EC:3.4.18.1]
103542159  NAPSA; napsin A aspartic peptidase [KO:K08565] [EC:3.4.23.-]
103556526  LGMN; legumain [KO:K01369] [EC:3.4.22.34]
103553536  TPP1; tripeptidyl peptidase 1 [KO:K01279] [EC:3.4.14.9]
103542795  GLA; galactosidase alpha [KO:K01189] [EC:3.2.1.22]
103561107  GLB1; galactosidase beta 1 [KO:K12309] [EC:3.2.1.23]
103547327  GAA; glucosidase alpha, acid [KO:K12316] [EC:3.2.1.20]
103552844  GBA; glucosylceramidase beta [KO:K01201] [EC:3.2.1.45]
103565375  IDUA; iduronidase, alpha-L- [KO:K01217] [EC:3.2.1.76]
103541990  NAGA; alpha-N-acetylgalactosaminidase [KO:K01204] [EC:3.2.1.49]
103550388  NAGLU; N-acetyl-alpha-glucosaminidase [KO:K01205] [EC:3.2.1.50]
103551800  GALC; galactosylceramidase [KO:K01202] [EC:3.2.1.46]
103558035  GUSB; glucuronidase beta [KO:K01195] [EC:3.2.1.31]
103558522  FUCA2; fucosidase, alpha-L- 2, plasma [KO:K01206] [EC:3.2.1.51]
103540706  FUCA1; fucosidase, alpha-L- 1, tissue [KO:K01206] [EC:3.2.1.51]
103555469  HEXA; hexosaminidase subunit alpha [KO:K12373] [EC:3.2.1.52]
103541849  HEXB; hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
103556721  MANBA; mannosidase beta [KO:K01192] [EC:3.2.1.25]
103541297  beta-mannosidase-like [KO:K01192] [EC:3.2.1.25]
103545166  lysosomal alpha-mannosidase-like [KO:K12311] [EC:3.2.1.24]
103542101  lysosomal alpha-mannosidase-like [KO:K12311] [EC:3.2.1.24]
103563289  NEU1; neuraminidase 1 [KO:K01186] [EC:3.2.1.18]
103544212  SPAM1; sperm adhesion molecule 1 [KO:K01197] [EC:3.2.1.35]
103557348  HYAL3; hyaluronoglucosaminidase 3 [KO:K01197] [EC:3.2.1.35]
103557349  hyaluronidase-2 [KO:K01197] [EC:3.2.1.35]
103560648  HYAL4; hyaluronoglucosaminidase 4 [KO:K01197] [EC:3.2.1.35]
103540526  ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
103548666  ARSB; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
103558473  ARSG; arylsulfatase G [KO:K12381] [EC:3.1.6.-]
103541880  GALNS; galactosamine (N-acetyl)-6-sulfatase [KO:K01132] [EC:3.1.6.4]
103557809  GNS; glucosamine (N-acetyl)-6-sulfatase [KO:K01137] [EC:3.1.6.14]
103540044  IDS; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
103547325  SGSH; N-sulfoglucosamine sulfohydrolase [KO:K01565] [EC:3.10.1.1]
103566588  LIPA; lipase A, lysosomal acid type [KO:K01052] [EC:3.1.1.13]
103549713  PLA2G15; phospholipase A2 group XV [KO:K06129] [EC:3.1.1.5]
103557287  DNASE2B; deoxyribonuclease 2 beta [KO:K01158] [EC:3.1.22.1]
103540639  DNASE2; deoxyribonuclease 2, lysosomal [KO:K01158] [EC:3.1.22.1]
103566744  ACP2; acid phosphatase 2, lysosomal [KO:K14410] [EC:3.1.3.2]
103562319  ACP5; acid phosphatase 5, tartrate resistant [KO:K14379] [EC:3.1.3.2]
103553547  SMPD1; sphingomyelin phosphodiesterase 1 [KO:K12350] [EC:3.1.4.12]
103556309  ASAH1; N-acylsphingosine amidohydrolase 1 [KO:K12348] [EC:3.5.1.23]
103544136  AGA; aspartylglucosaminidase [KO:K01444] [EC:3.5.1.26]
103549406  PSAP; prosaposin [KO:K12382]
103553896  PSAPL1; prosaposin-like 1 [KO:K12382]
103549097  GM2A; GM2 ganglioside activator [KO:K12383]
103553563  PPT1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
103566642  PPT2; palmitoyl-protein thioesterase 2 [KO:K01074] [EC:3.1.2.22]
103549667  LAMP1; lysosomal associated membrane protein 1 [KO:K06528]
103564255  LAMP2; lysosomal associated membrane protein 2 [KO:K06528]
103558193  LAMP3; lysosomal associated membrane protein 3 [KO:K06562]
103557712  CD68; CD68 molecule [KO:K06501]
103560205  CD63; CD63 molecule [KO:K06497]
103562214  SCARB2; scavenger receptor class B member 2 [KO:K12384]
103561501  NPC1; NPC intracellular cholesterol transporter 1 [KO:K12385]
103548599  NPC2; NPC intracellular cholesterol transporter 2 [KO:K13443]
103562930  CTNS; cystinosin, lysosomal cystine transporter [KO:K12386]
103543123  sialin-like [KO:K12301]
103564154  SLC11A1; solute carrier family 11 member 1 [KO:K12347]
103545846  SLC11A2; solute carrier family 11 member 2 [KO:K21398]
103541283  LAPTM4B; lysosomal protein transmembrane 4 beta [KO:K12387]
103560370  LAPTM5; lysosomal protein transmembrane 5 [KO:K12387]
103556436  LAPTM4A; lysosomal protein transmembrane 4 alpha [KO:K12387]
103568012  ABCA2; ATP binding cassette subfamily A member 2 [KO:K05642]
103549876  ABCB9; ATP binding cassette subfamily B member 9 [KO:K05656]
103550751  ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 [KO:K12305] [EC:3.6.1.6]
103553061  SORT1; sortilin 1 [KO:K12388]
103562739  CLN3; CLN3, battenin [KO:K12389]
103554778  CLN5; ceroid-lipofuscinosis, neuronal 5 [KO:K12390]
103562824  MFSD8; major facilitator superfamily domain containing 8 [KO:K12307]
103540950  HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
103550290  SUMF1; sulfatase modifying factor 1 [KO:K13444] [EC:1.8.3.7]
103555699  GNPTAB; N-acetylglucosamine-1-phosphate transferase alpha and beta subunits [KO:K08239] [EC:2.7.8.17]
103559113  GNPTG; N-acetylglucosamine-1-phosphate transferase gamma subunit [KO:K10087]
103563128  NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
103558448  IGF2R; insulin like growth factor 2 receptor [KO:K06564]
103542483  M6PR; mannose-6-phosphate receptor, cation dependent [KO:K10089]
103564405  CLTA; clathrin light chain A [KO:K04644]
103563364  CLTB; clathrin light chain B [KO:K04645]
103542958  clathrin heavy chain 2-like [KO:K04646]
103565379  CLTC; clathrin heavy chain [KO:K04646]
103551741  AP1G1; adaptor related protein complex 1 gamma 1 subunit [KO:K12391]
103563007  AP1G2; adaptor related protein complex 1 gamma 2 subunit [KO:K12391]
103548098  AP1B1; adaptor related protein complex 1 beta 1 subunit [KO:K12392]
103558370  AP1M1; adaptor related protein complex 1 mu 1 subunit [KO:K12393]
103562419  AP1M2; adaptor related protein complex 1 mu 2 subunit [KO:K12393]
103541184  AP1S1; adaptor related protein complex 1 sigma 1 subunit [KO:K12394]
103543754  AP-1 complex subunit sigma-2 [KO:K12394]
103550310  AP1S3; adaptor related protein complex 1 sigma 3 subunit [KO:K12395]
103542499  AP-3 complex subunit delta-1-like [KO:K12396]
103546245  AP3B2; adaptor related protein complex 3 beta 2 subunit [KO:K12397]
103548660  AP3B1; adaptor related protein complex 3 beta 1 subunit [KO:K12397]
103549471  AP3M1; adaptor related protein complex 3 mu 1 subunit [KO:K12398]
103562904  AP3M2; adaptor related protein complex 3 mu 2 subunit [KO:K12398]
103546966  AP3S1; adaptor related protein complex 3 sigma 1 subunit [KO:K12399]
103558653  AP-3 complex subunit sigma-2 [KO:K12399]
103546928  AP4E1; adaptor related protein complex 4 epsilon 1 subunit [KO:K12400]
103565932  AP4B1; adaptor related protein complex 4 beta 1 subunit [KO:K12401]
103561773  AP4M1; adaptor related protein complex 4 mu 1 subunit [KO:K12402]
103552714  AP4S1; adaptor related protein complex 4 sigma 1 subunit [KO:K12403]
103546574  GGA3; golgi associated, gamma adaptin ear containing, ARF binding protein 3 [KO:K12404]
103562665  GGA2; golgi associated, gamma adaptin ear containing, ARF binding protein 2 [KO:K12404]
103543192  GGA1; golgi associated, gamma adaptin ear containing, ARF binding protein 1 [KO:K12404]
103540431  MCOLN1; mucolipin 1 [KO:K04992]
103557145  LITAF; lipopolysaccharide induced TNF factor [KO:K19363]
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   
LinkDB

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