KEGG   PATHWAY: ppad04142
Entry
ppad04142                   Pathway                                
Name
Lysosome - Panthera pardus (leopard)
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
ppad04142  Lysosome
ppad04142

Other DBs
GO: 0005764
Organism
Panthera pardus (leopard) [GN:ppad]
Gene
109245120  ACP2; lysosomal acid phosphatase isoform X1 [KO:K14410] [EC:3.1.3.2]
109245394  IGF2R; cation-independent mannose-6-phosphate receptor [KO:K06564]
109245536  GALNS; N-acetylgalactosamine-6-sulfatase [KO:K01132] [EC:3.1.6.4]
109245605  TLDC1; TLD domain-containing protein 1 isoform X1 [KO:K27947]
109245944  CD68; macrosialin [KO:K06501]
109246178  DNASE2B; deoxyribonuclease-2-beta isoform X1 [KO:K01158] [EC:3.1.22.1]
109246765  CTSW; cathepsin W isoform X1 [KO:K08569] [EC:3.4.22.-]
109246807  CTSF; cathepsin F [KO:K01373] [EC:3.4.22.41]
109246996  TCIRG1; LOW QUALITY PROTEIN: V-type proton ATPase 116 kDa subunit a [KO:K02154]
109247424  MANBA; beta-mannosidase isoform X1 [KO:K01192] [EC:3.2.1.25]
109247536  AP1M1; AP-1 complex subunit mu-1 isoform X1 [KO:K12393]
109247837  [KO:K01197] [EC:3.2.1.35]
109247842  HYAL3; hyaluronidase-3 [KO:K01197] [EC:3.2.1.35]
109248231  SLC17A5; sialin isoform X1 [KO:K12301]
109248325  AP1S2; AP-1 complex subunit sigma-2 isoform X1 [KO:K12394]
109248507  NCOA7; nuclear receptor coactivator 7 isoform X1 [KO:K25442]
109248704  GLB1; beta-galactosidase isoform X1 [KO:K12309] [EC:3.2.1.23]
109249112  SLC11A2; natural resistance-associated macrophage protein 2 isoform X1 [KO:K21398]
109249123  MFSD8; major facilitator superfamily domain-containing protein 8 isoform X1 [KO:K12307]
109250527  AP1S1; AP-1 complex subunit sigma-1A [KO:K12394]
109250657  GUSB; beta-glucuronidase isoform X1 [KO:K01195] [EC:3.2.1.31]
109250737  LAMP2; LOW QUALITY PROTEIN: lysosome-associated membrane glycoprotein 2 [KO:K06528]
109251266  AP4M1; AP-4 complex subunit mu-1 isoform X1 [KO:K12402]
109251630  [KO:K01365] [EC:3.4.22.15]
109251669  [KO:K01365] [EC:3.4.22.15]
109251670  [KO:K01365] [EC:3.4.22.15]
109251671  [KO:K01365] [EC:3.4.22.15]
109251673  [KO:K01365] [EC:3.4.22.15]
109251674  [KO:K01365] [EC:3.4.22.15]
109251680  [KO:K01365] [EC:3.4.22.15]
109251681  [KO:K01365] [EC:3.4.22.15]
109252530  FUCA2; plasma alpha-L-fucosidase isoform X1 [KO:K01206] [EC:3.2.1.51]
109253017  NAPSA; napsin-A [KO:K08565] [EC:3.4.23.-]
109253464  CTSZ; cathepsin Z [KO:K08568] [EC:3.4.18.1]
109253562  AP1B1; AP-1 complex subunit beta-1 isoform X1 [KO:K12392]
109253830  NPC2; epididymal secretory protein E1 [KO:K13443]
109253930  MAN2B1; LOW QUALITY PROTEIN: lysosomal alpha-mannosidase [KO:K12311] [EC:3.2.1.24]
109254029  DNASE2; LOW QUALITY PROTEIN: deoxyribonuclease-2-alpha [KO:K01158] [EC:3.1.22.1]
109254055  ACP5; tartrate-resistant acid phosphatase type 5 [KO:K14379] [EC:3.1.3.2]
109254101  AP1M2; AP-1 complex subunit mu-2 [KO:K12393]
109254182  MCOLN1; mucolipin-1 isoform X1 [KO:K04992]
109254292  AP4S1; AP-4 complex subunit sigma-1 isoform X1 [KO:K12403]
109254651  SUMF1; sulfatase-modifying factor 1 isoform X1 [KO:K13444] [EC:1.8.3.7]
109254798  ATP6V0B; V-type proton ATPase 21 kDa proteolipid subunit isoform X1 [KO:K03661]
109254853  PPT1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
109254958  [KO:K01319] [EC:3.4.21.20]
109255011  AP1G2; AP-1 complex subunit gamma-like 2 isoform X1 [KO:K12391]
109255108  CTNS; cystinosin isoform X1 [KO:K12386]
109255300  LAPTM5; lysosomal-associated transmembrane protein 5 [KO:K12387]
109255402  LIPA; lysosomal acid lipase/cholesteryl ester hydrolase [KO:K01052] [EC:3.1.1.13]
109255571  GGA2; ADP-ribosylation factor-binding protein GGA2 [KO:K12404]
109256112  CLTC; clathrin heavy chain 1 [KO:K04646]
109256251  GBA; glucosylceramidase isoform X1 [KO:K01201] [EC:3.2.1.45]
109256525  CLN3; battenin [KO:K12389]
109257023  CTSK; cathepsin K isoform X1 [KO:K01371] [EC:3.4.22.38]
109257065  CTSS; cathepsin S isoform X1 [KO:K01368] [EC:3.4.22.27]
109257566  CTSE; cathepsin E isoform X1 [KO:K01382] [EC:3.4.23.34]
109257712  AP3D1; AP-3 complex subunit delta-1 isoform X1 [KO:K12396]
109257861  AGA; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase [KO:K01444] [EC:3.5.1.26]
109258436  ATP6V1H; V-type proton ATPase subunit H isoform X1 [KO:K02144]
109258538  ATP6V0A1; V-type proton ATPase 116 kDa subunit a isoform X1 [KO:K02154]
109258566  NAGLU; alpha-N-acetylglucosaminidase [KO:K01205] [EC:3.2.1.50]
109258633  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
109258697  IDS; LOW QUALITY PROTEIN: iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
109258754  ABCA2; ATP-binding cassette sub-family A member 2 [KO:K05642]
109258839  GLA; alpha-galactosidase A isoform X1 [KO:K01189] [EC:3.2.1.22]
109259300  IDUA; alpha-L-iduronidase isoform X1 [KO:K01217] [EC:3.2.1.76]
109259409  CTSD; LOW QUALITY PROTEIN: cathepsin D [KO:K01379] [EC:3.4.23.5]
109260509  NEU1; sialidase-1 [KO:K01186] [EC:3.2.1.18]
109260524  PPT2; lysosomal thioesterase PPT2 [KO:K01074] [EC:3.1.2.22]
109260771  [KO:K01365] [EC:3.4.22.15]
109261151  ATP6AP1; V-type proton ATPase subunit S1 isoform X1 [KO:K03662]
109262246  HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
109262270  AP3M2; AP-3 complex subunit mu-2 [KO:K12398]
109262395  CTSB; cathepsin B isoform X1 [KO:K01363] [EC:3.4.22.1]
109262485  CTSO; cathepsin O [KO:K01374] [EC:3.4.22.42]
109262730  HEXA; beta-hexosaminidase subunit alpha isoform X1 [KO:K12373] [EC:3.2.1.52]
109262987  DMXL2; dmX-like protein 2 isoform X1 [KO:K24155]
109262994  AP4E1; AP-4 complex subunit epsilon-1 isoform X1 [KO:K12400]
109263522  CD164; sialomucin core protein 24 [KO:K06546]
109264034  LAMP1; lysosome-associated membrane glycoprotein 1 [KO:K06528]
109264204  CLN5; ceroid-lipofuscinosis neuronal protein 5 [KO:K12390]
109264383  PSAP; prosaposin isoform X1 [KO:K12382]
109264430  AP3M1; AP-3 complex subunit mu-1 [KO:K12398]
109264988  AP1S3; AP-1 complex subunit sigma-3 isoform X1 [KO:K12395]
109265070  SLC11A1; natural resistance-associated macrophage protein 1 [KO:K12347]
109265406  GNPTAB; N-acetylglucosamine-1-phosphotransferase subunits alpha/beta isoform X1 [KO:K08239] [EC:2.7.8.17]
109265540  GGA1; ADP-ribosylation factor-binding protein GGA1 isoform X1 [KO:K12404]
109265652  NAGA; alpha-N-acetylgalactosaminidase [KO:K01204] [EC:3.2.1.49]
109265790  ARSA; arylsulfatase A isoform X1 [KO:K01134] [EC:3.1.6.8]
109267324  CTSC; dipeptidyl peptidase 1 isoform X1 [KO:K01275] [EC:3.4.14.1]
109267551  CLTB; clathrin light chain B isoform X1 [KO:K04645]
109268038  LAPTM4A; lysosomal-associated transmembrane protein 4A [KO:K12387]
109268920  HYAL4; hyaluronidase-4 [KO:K01197] [EC:3.2.1.35]
109268922  SPAM1; hyaluronidase PH-20 [KO:K01197] [EC:3.2.1.35]
109269038  [KO:K01197] [EC:3.2.1.35]
109269076  ATP6V0A4; V-type proton ATPase 116 kDa subunit a [KO:K02154]
109269441  ATP6V0D2; V-type proton ATPase subunit d 2 [KO:K02146]
109270144  CLTA; clathrin light chain A isoform X1 [KO:K04644]
109270295  CD63; CD63 antigen [KO:K06497]
109270435  GNS; N-acetylglucosamine-6-sulfatase [KO:K01137] [EC:3.1.6.14]
109270670  AP1G1; AP-1 complex subunit gamma-1 isoform X1 [KO:K12391]
109270737  PLA2G15; group XV phospholipase A2 isoform X1 [KO:K06129] [EC:3.1.1.5]
109270766  ATP6V0D1; V-type proton ATPase subunit d 1 [KO:K02146]
109271061  SMPD1; sphingomyelin phosphodiesterase isoform X1 [KO:K12350] [EC:3.1.4.12]
109271080  TPP1; tripeptidyl-peptidase 1 isoform X1 [KO:K01279] [EC:3.4.14.9]
109271987  SORT1; sortilin isoform X1 [KO:K12388]
109272084  AP4B1; AP-4 complex subunit beta-1 isoform X1 [KO:K12401]
109272351  CTSA; lysosomal protective protein isoform X1 [KO:K13289] [EC:3.4.16.5]
109273446  [KO:K12399]
109273509  AP3B2; AP-3 complex subunit beta-2 isoform X1 [KO:K12397]
109273576  CTSH; pro-cathepsin H [KO:K01366] [EC:3.4.22.16]
109274307  FUCA1; tissue alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
109274421  GALC; galactocerebrosidase isoform X1 [KO:K01202] [EC:3.2.1.46]
109274478  LGMN; legumain [KO:K01369] [EC:3.4.22.34]
109274943  [KO:K01135] [EC:3.1.6.12]
109274954  AP3B1; AP-3 complex subunit beta-1 [KO:K12397]
109275001  HEXB; beta-hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
109275198  LAMP3; lysosome-associated membrane glycoprotein 3 isoform X1 [KO:K06562]
109275681  WDR7; WD repeat-containing protein 7 isoform X1 [KO:K24738]
109275980  GM2A; ganglioside GM2 activator [KO:K12383]
109276053  M6PR; cation-dependent mannose-6-phosphate receptor [KO:K10089]
109276490  NPC1; Niemann-Pick C1 protein isoform X1 [KO:K12385]
109276815  SGSH; N-sulphoglucosamine sulphohydrolase [KO:K01565] [EC:3.10.1.1]
109276817  GAA; LOW QUALITY PROTEIN: lysosomal alpha-glucosidase [KO:K12316] [EC:3.2.1.20]
109276941  GGA3; ADP-ribosylation factor-binding protein GGA3 isoform X1 [KO:K12404]
109277041  ARSG; arylsulfatase G isoform X1 [KO:K12381] [EC:3.1.6.-]
109277196  ASAH1; acid ceramidase [KO:K12348] [EC:3.5.1.23]
109277329  ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 isoform X1 [KO:K12305] [EC:3.6.1.6]
109277425  SCARB2; lysosome membrane protein 2 [KO:K12384]
109277725  ABCB9; ATP-binding cassette sub-family B member 9 isoform X1 [KO:K05656]
109277749  ATP6V0A2; V-type proton ATPase 116 kDa subunit a isoform X1 [KO:K02154]
109278038  LAPTM4B; lysosomal-associated transmembrane protein 4B [KO:K12387]
109278097  GNPTG; N-acetylglucosamine-1-phosphotransferase subunit gamma [KO:K10087]
109278173  ATP6V0C; V-type proton ATPase 16 kDa proteolipid subunit isoform X1 [KO:K02155]
109278286  NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase isoform X1 [KO:K01125] [EC:3.1.4.45]
109278300  [KO:K19363]
109278331  LITAF; lipopolysaccharide-induced tumor necrosis factor-alpha factor [KO:K19363]
109278894  DMXL1; dmX-like protein 1 isoform X1 [KO:K24155]
109278918  AP3S1; AP-3 complex subunit sigma-1 isoform X1 [KO:K12399]
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 22:339-352 (2022)
DOI:10.1038/s41577-021-00626-8
Related
pathway
ppad00511  Other glycan degradation
ppad00531  Glycosaminoglycan degradation
ppad04130  SNARE interactions in vesicular transport
ppad04140  Autophagy - animal
ppad04144  Endocytosis
KO pathway
ko04142   

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