KEGG   PATHWAY: hsa04142
Entry
hsa04142                    Pathway                                
Name
Lysosome - Homo sapiens (human)
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
hsa04142  Lysosome
hsa04142

Drug
D02784  Agalsidase alfa (USAN/INN)
D02810  Alglucerase (JAN/USAN/INN)
D03020  Imiglucerase (USAN/INN)
D03207  Alglucosidase alfa (USAN/INN)
D03228  Agalsidase beta (INN)
D04499  Idursulfase (USAN/INN)
D04670  Laronidase (USAN)
D05031  Migalastat hydrochloride (JAN/USAN)
D06565  Galsulfase (USAN/INN)
D08955  Odanacatib (JAN/USAN)
D09029  Velaglucerase alfa (USAN)
D09605  Duvoglustat (USAN/INN)
D09606  Duvoglustat hydrochloride (USAN)
D09675  Taliglucerase alfa (USAN/INN)
D10333  Elosulfase alfa (USAN)
D10377  Sebelipase alfa (USAN)
D10813  Cerliponase alfa (USAN/INN)
D10820  Olipudase alfa (USAN/INN)
D11004  Vestronidase alfa (USAN/INN)
D11024  Velmanase alfa (USAN/INN)
D11025  Idursulfase beta (INN)
D11685  Pegunigalsidase alfa (USAN/INN)
D11744  Avalglucosidase alfa (USAN/INN)
D11798  Cipaglucosidase alfa (USAN)
D11812  Pabinafusp alfa (genetical recombination) (JAN)
D11828  Clervonafusp alfa (USAN/INN)
D12120  Brensocatib (USAN/INN)
D12462  Cebsulfase alfa (USAN/INN)
D12580  Sudotaxel zendusortide (USAN/INN)
D12581  Zendusortide (USAN/INN)
D12746  Brensocatib hydrate (JAN)
D12806  Tividenofusp alfa (USAN/INN)
D12877  Atidarsagene autotemcel (USAN/INN)
Other DBs
GO: 0005764
Organism
Homo sapiens (human) [GN:hsa]
Gene
10053  AP1M2; adaptor related protein complex 1 subunit mu 2 [KO:K12393]
10239  AP3S2; adaptor related protein complex 3 subunit sigma 2 [KO:K12399]
10312  TCIRG1; T cell immune regulator 1, ATPase H+ transporting V0 subunit a3 [KO:K02154]
10577  NPC2; NPC intracellular cholesterol transporter 2 [KO:K13443]
10717  AP4B1; adaptor related protein complex 4 subunit beta 1 [KO:K12401]
1075  CTSC; cathepsin C [KO:K01275] [EC:3.4.14.1]
10947  AP3M2; adaptor related protein complex 3 subunit mu 2 [KO:K12398]
11154  AP4S1; adaptor related protein complex 4 subunit sigma 1 [KO:K12403]
1174  AP1S1; adaptor related protein complex 1 subunit sigma 1 [KO:K12394]
1176  AP3S1; adaptor related protein complex 3 subunit sigma 1 [KO:K12399]
1200  TPP1; tripeptidyl peptidase 1 [KO:K01279] [EC:3.4.14.9]
1201  CLN3; CLN3 lysosomal/endosomal transmembrane protein, battenin [KO:K12389]
1203  CLN5; CLN5 intracellular trafficking protein [KO:K12390]
1211  CLTA; clathrin light chain A [KO:K04644]
1212  CLTB; clathrin light chain B [KO:K04645]
1213  CLTC; clathrin heavy chain [KO:K04646]
130340  AP1S3; adaptor related protein complex 1 subunit sigma 3 [KO:K12395]
135112  NCOA7; nuclear receptor coactivator 7 [KO:K25442]
138050  HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
1497  CTNS; cystinosin, lysosomal cystine transporter [KO:K12386]
1508  CTSB; cathepsin B [KO:K01363] [EC:3.4.22.1]
1509  CTSD; cathepsin D [KO:K01379] [EC:3.4.23.5]
1510  CTSE; cathepsin E [KO:K01382] [EC:3.4.23.34]
1511  CTSG; cathepsin G [KO:K01319] [EC:3.4.21.20]
1512  CTSH; cathepsin H [KO:K01366] [EC:3.4.22.16]
1513  CTSK; cathepsin K [KO:K01371] [EC:3.4.22.38]
1514  CTSL; cathepsin L [KO:K01365] [EC:3.4.22.15]
1515  CTSV; cathepsin V [KO:K01375] [EC:3.4.22.43]
1519  CTSO; cathepsin O [KO:K01374] [EC:3.4.22.42]
1520  CTSS; cathepsin S [KO:K01368] [EC:3.4.22.27]
1521  CTSW; cathepsin W [KO:K08569] [EC:3.4.22.-]
1522  CTSZ; cathepsin Z [KO:K08568] [EC:3.4.18.1]
162  AP1B1; adaptor related protein complex 1 subunit beta 1 [KO:K12392]
164  AP1G1; adaptor related protein complex 1 subunit gamma 1 [KO:K12391]
1657  DMXL1; Dmx like 1 [KO:K24155]
175  AGA; aspartylglucosaminidase [KO:K01444] [EC:3.5.1.26]
1777  DNASE2; deoxyribonuclease 2, lysosomal [KO:K01158] [EC:3.1.22.1]
20  ABCA2; ATP binding cassette subfamily A member 2 [KO:K05642]
22901  ARSG; arylsulfatase G [KO:K12381] [EC:3.1.6.-]
23062  GGA2; golgi associated, gamma adaptin ear containing, ARF binding protein 2 [KO:K12404]
23163  GGA3; golgi associated, gamma adaptin ear containing, ARF binding protein 3 [KO:K12404]
23312  DMXL2; Dmx like 2 [KO:K24155]
23335  WDR7; WD repeat domain 7 [KO:K24738]
23431  AP4E1; adaptor related protein complex 4 subunit epsilon 1 [KO:K12400]
23457  ABCB9; ATP binding cassette subfamily B member 9 [KO:K05656]
23545  ATP6V0A2; ATPase H+ transporting V0 subunit a2 [KO:K02154]
23553  HYAL4; hyaluronidase 4 [KO:K01197] [EC:3.2.1.35]
23659  PLA2G15; phospholipase A2 group XV [KO:K06129] [EC:3.1.1.5]
245972  ATP6V0D2; ATPase H+ transporting V0 subunit d2 [KO:K02146]
2517  FUCA1; alpha-L-fucosidase 1 [KO:K01206] [EC:3.2.1.51]
2519  FUCA2; alpha-L-fucosidase 2 [KO:K01206] [EC:3.2.1.51]
2548  GAA; alpha glucosidase [KO:K12316] [EC:3.2.1.20]
256471  MFSD8; major facilitator superfamily domain containing 8 [KO:K12307]
2581  GALC; galactosylceramidase [KO:K01202] [EC:3.2.1.46]
2588  GALNS; galactosamine (N-acetyl)-6-sulfatase [KO:K01132] [EC:3.1.6.4]
26088  GGA1; golgi associated, gamma adaptin ear containing, ARF binding protein 1 [KO:K12404]
2629  GBA1; glucosylceramidase beta 1 [KO:K01201] [EC:3.2.1.45]
26503  SLC17A5; solute carrier family 17 member 5 [KO:K12301]
26985  AP3M1; adaptor related protein complex 3 subunit mu 1 [KO:K12398]
27074  LAMP3; lysosomal associated membrane protein 3 [KO:K06562]
2717  GLA; galactosidase alpha [KO:K01189] [EC:3.2.1.22]
2720  GLB1; galactosidase beta 1 [KO:K12309] [EC:3.2.1.23]
2760  GM2A; ganglioside GM2 activator [KO:K12383]
2799  GNS; glucosamine (N-acetyl)-6-sulfatase [KO:K01137] [EC:3.1.6.14]
285362  SUMF1; sulfatase modifying factor 1 [KO:K13444] [EC:1.8.3.7]
2990  GUSB; glucuronidase beta [KO:K01195] [EC:3.2.1.31]
3073  HEXA; hexosaminidase subunit alpha [KO:K12373] [EC:3.2.1.52]
3074  HEXB; hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
3373  HYAL1; hyaluronidase 1 [KO:K01197] [EC:3.2.1.35]
3423  IDS; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
3425  IDUA; alpha-L-iduronidase [KO:K01217] [EC:3.2.1.76]
3482  IGF2R; insulin like growth factor 2 receptor [KO:K06564]
3916  LAMP1; lysosomal associated membrane protein 1 [KO:K06528]
3920  LAMP2; lysosomal associated membrane protein 2 [KO:K06528]
3988  LIPA; lipase A, lysosomal acid type [KO:K01052] [EC:3.1.1.13]
4074  M6PR; mannose-6-phosphate receptor, cation dependent [KO:K10089]
410  ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
411  ARSB; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
4125  MAN2B1; mannosidase alpha class 2B member 1 [KO:K12311] [EC:3.2.1.24]
4126  MANBA; mannosidase beta [KO:K01192] [EC:3.2.1.25]
427  ASAH1; N-acylsphingosine amidohydrolase 1 [KO:K12348] [EC:3.5.1.23]
4668  NAGA; alpha-N-acetylgalactosaminidase [KO:K01204] [EC:3.2.1.49]
4669  NAGLU; N-acetyl-alpha-glucosaminidase [KO:K01205] [EC:3.2.1.50]
4758  NEU1; neuraminidase 1 [KO:K01186] [EC:3.2.1.18]
4864  NPC1; NPC intracellular cholesterol transporter 1 [KO:K12385]
4891  SLC11A2; solute carrier family 11 member 2 [KO:K21398]
50617  ATP6V0A4; ATPase H+ transporting V0 subunit a4 [KO:K02154]
51172  NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
51606  ATP6V1H; ATPase H+ transporting V1 subunit H [KO:K02144]
527  ATP6V0C; ATPase H+ transporting V0 subunit c [KO:K02155]
53  ACP2; acid phosphatase 2, lysosomal [KO:K14410] [EC:3.1.3.2]
533  ATP6V0B; ATPase H+ transporting V0 subunit b [KO:K03661]
535  ATP6V0A1; ATPase H+ transporting V0 subunit a1 [KO:K02154]
537  ATP6AP1; ATPase H+ transporting accessory protein 1 [KO:K03662]
54  ACP5; acid phosphatase 5, tartrate resistant [KO:K14379] [EC:3.1.3.2]
5476  CTSA; cathepsin A [KO:K13289] [EC:3.4.16.5]
55353  LAPTM4B; lysosomal protein transmembrane 4 beta [KO:K12387]
5538  PPT1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
5641  LGMN; legumain [KO:K01369] [EC:3.4.22.34]
5660  PSAP; prosaposin [KO:K12382]
57192  MCOLN1; mucolipin TRP cation channel 1 [KO:K04992]
57707  MEAK7; MTOR associated protein, eak-7 homolog [KO:K27947]
58511  DNASE2B; deoxyribonuclease 2 beta [KO:K01158] [EC:3.1.22.1]
6272  SORT1; sortilin 1 [KO:K12388]
6448  SGSH; N-sulfoglucosamine sulfohydrolase [KO:K01565] [EC:3.10.1.1]
6556  SLC11A1; solute carrier family 11 member 1 [KO:K12347]
6609  SMPD1; sphingomyelin phosphodiesterase 1 [KO:K12350] [EC:3.1.4.12]
6677  SPAM1; sperm adhesion molecule 1 [KO:K01197] [EC:3.2.1.35]
768239  PSAPL1; prosaposin like 1 [KO:K12382]
7805  LAPTM5; lysosomal protein transmembrane 5 [KO:K12387]
79158  GNPTAB; N-acetylglucosamine-1-phosphate transferase subunits alpha and beta [KO:K08239] [EC:2.7.8.17]
8120  AP3B2; adaptor related protein complex 3 subunit beta 2 [KO:K12397]
8218  CLTCL1; clathrin heavy chain like 1 [KO:K04646]
8372  HYAL3; hyaluronidase 3 [KO:K01197] [EC:3.2.1.35]
84572  GNPTG; N-acetylglucosamine-1-phosphate transferase subunit gamma [KO:K10087]
8546  AP3B1; adaptor related protein complex 3 subunit beta 1 [KO:K12397]
8692  HYAL2; hyaluronidase 2 [KO:K01197] [EC:3.2.1.35]
8722  CTSF; cathepsin F [KO:K01373] [EC:3.4.22.41]
8763  CD164; CD164 molecule [KO:K06546]
8905  AP1S2; adaptor related protein complex 1 subunit sigma 2 [KO:K12394]
8906  AP1G2; adaptor related protein complex 1 subunit gamma 2 [KO:K12391]
8907  AP1M1; adaptor related protein complex 1 subunit mu 1 [KO:K12393]
8943  AP3D1; adaptor related protein complex 3 subunit delta 1 [KO:K12396]
9114  ATP6V0D1; ATPase H+ transporting V0 subunit d1 [KO:K02146]
9179  AP4M1; adaptor related protein complex 4 subunit mu 1 [KO:K12402]
9374  PPT2; palmitoyl-protein thioesterase 2 [KO:K01074] [EC:3.1.2.22]
9476  NAPSA; napsin A aspartic peptidase [KO:K08565] [EC:3.4.23.-]
950  SCARB2; scavenger receptor class B member 2 [KO:K12384]
9516  LITAF; lipopolysaccharide induced TNF factor [KO:K19363]
9583  ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 [KO:K12305] [EC:3.6.1.6]
967  CD63; CD63 molecule [KO:K06497]
968  CD68; CD68 molecule [KO:K06501]
9741  LAPTM4A; lysosomal protein transmembrane 4 alpha [KO:K12387]
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
hsa00511  Other glycan degradation
hsa00531  Glycosaminoglycan degradation
hsa04130  SNARE interactions in vesicular transport
hsa04140  Autophagy - animal
hsa04144  Endocytosis
KO pathway
ko04142   

  All links  
No link information was found.
KEGG   PATHWAY: hsa04142
Entry
hsa04142                    Pathway                                
Name
Lysosome - Homo sapiens (human)
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
hsa04142  Lysosome
hsa04142

Drug
D02784  Agalsidase alfa (USAN/INN)
D02810  Alglucerase (JAN/USAN/INN)
D03020  Imiglucerase (USAN/INN)
D03207  Alglucosidase alfa (USAN/INN)
D03228  Agalsidase beta (INN)
D04499  Idursulfase (USAN/INN)
D04670  Laronidase (USAN)
D05031  Migalastat hydrochloride (JAN/USAN)
D06565  Galsulfase (USAN/INN)
D08955  Odanacatib (JAN/USAN)
D09029  Velaglucerase alfa (USAN)
D09605  Duvoglustat (USAN/INN)
D09606  Duvoglustat hydrochloride (USAN)
D09675  Taliglucerase alfa (USAN/INN)
D10333  Elosulfase alfa (USAN)
D10377  Sebelipase alfa (USAN)
D10813  Cerliponase alfa (USAN/INN)
D10820  Olipudase alfa (USAN/INN)
D11004  Vestronidase alfa (USAN/INN)
D11024  Velmanase alfa (USAN/INN)
D11025  Idursulfase beta (INN)
D11685  Pegunigalsidase alfa (USAN/INN)
D11744  Avalglucosidase alfa (USAN/INN)
D11798  Cipaglucosidase alfa (USAN)
D11812  Pabinafusp alfa (genetical recombination) (JAN)
D11828  Clervonafusp alfa (USAN/INN)
D12120  Brensocatib (USAN/INN)
D12462  Cebsulfase alfa (USAN/INN)
D12580  Sudotaxel zendusortide (USAN/INN)
D12581  Zendusortide (USAN/INN)
D12746  Brensocatib hydrate (JAN)
D12806  Tividenofusp alfa (USAN/INN)
D12877  Atidarsagene autotemcel (USAN/INN)
Other DBs
GO: 0005764
Organism
Homo sapiens (human) [GN:hsa]
Gene
10053  AP1M2; adaptor related protein complex 1 subunit mu 2 [KO:K12393]
10239  AP3S2; adaptor related protein complex 3 subunit sigma 2 [KO:K12399]
10312  TCIRG1; T cell immune regulator 1, ATPase H+ transporting V0 subunit a3 [KO:K02154]
10577  NPC2; NPC intracellular cholesterol transporter 2 [KO:K13443]
10717  AP4B1; adaptor related protein complex 4 subunit beta 1 [KO:K12401]
1075  CTSC; cathepsin C [KO:K01275] [EC:3.4.14.1]
10947  AP3M2; adaptor related protein complex 3 subunit mu 2 [KO:K12398]
11154  AP4S1; adaptor related protein complex 4 subunit sigma 1 [KO:K12403]
1174  AP1S1; adaptor related protein complex 1 subunit sigma 1 [KO:K12394]
1176  AP3S1; adaptor related protein complex 3 subunit sigma 1 [KO:K12399]
1200  TPP1; tripeptidyl peptidase 1 [KO:K01279] [EC:3.4.14.9]
1201  CLN3; CLN3 lysosomal/endosomal transmembrane protein, battenin [KO:K12389]
1203  CLN5; CLN5 intracellular trafficking protein [KO:K12390]
1211  CLTA; clathrin light chain A [KO:K04644]
1212  CLTB; clathrin light chain B [KO:K04645]
1213  CLTC; clathrin heavy chain [KO:K04646]
130340  AP1S3; adaptor related protein complex 1 subunit sigma 3 [KO:K12395]
135112  NCOA7; nuclear receptor coactivator 7 [KO:K25442]
138050  HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
1497  CTNS; cystinosin, lysosomal cystine transporter [KO:K12386]
1508  CTSB; cathepsin B [KO:K01363] [EC:3.4.22.1]
1509  CTSD; cathepsin D [KO:K01379] [EC:3.4.23.5]
1510  CTSE; cathepsin E [KO:K01382] [EC:3.4.23.34]
1511  CTSG; cathepsin G [KO:K01319] [EC:3.4.21.20]
1512  CTSH; cathepsin H [KO:K01366] [EC:3.4.22.16]
1513  CTSK; cathepsin K [KO:K01371] [EC:3.4.22.38]
1514  CTSL; cathepsin L [KO:K01365] [EC:3.4.22.15]
1515  CTSV; cathepsin V [KO:K01375] [EC:3.4.22.43]
1519  CTSO; cathepsin O [KO:K01374] [EC:3.4.22.42]
1520  CTSS; cathepsin S [KO:K01368] [EC:3.4.22.27]
1521  CTSW; cathepsin W [KO:K08569] [EC:3.4.22.-]
1522  CTSZ; cathepsin Z [KO:K08568] [EC:3.4.18.1]
162  AP1B1; adaptor related protein complex 1 subunit beta 1 [KO:K12392]
164  AP1G1; adaptor related protein complex 1 subunit gamma 1 [KO:K12391]
1657  DMXL1; Dmx like 1 [KO:K24155]
175  AGA; aspartylglucosaminidase [KO:K01444] [EC:3.5.1.26]
1777  DNASE2; deoxyribonuclease 2, lysosomal [KO:K01158] [EC:3.1.22.1]
20  ABCA2; ATP binding cassette subfamily A member 2 [KO:K05642]
22901  ARSG; arylsulfatase G [KO:K12381] [EC:3.1.6.-]
23062  GGA2; golgi associated, gamma adaptin ear containing, ARF binding protein 2 [KO:K12404]
23163  GGA3; golgi associated, gamma adaptin ear containing, ARF binding protein 3 [KO:K12404]
23312  DMXL2; Dmx like 2 [KO:K24155]
23335  WDR7; WD repeat domain 7 [KO:K24738]
23431  AP4E1; adaptor related protein complex 4 subunit epsilon 1 [KO:K12400]
23457  ABCB9; ATP binding cassette subfamily B member 9 [KO:K05656]
23545  ATP6V0A2; ATPase H+ transporting V0 subunit a2 [KO:K02154]
23553  HYAL4; hyaluronidase 4 [KO:K01197] [EC:3.2.1.35]
23659  PLA2G15; phospholipase A2 group XV [KO:K06129] [EC:3.1.1.5]
245972  ATP6V0D2; ATPase H+ transporting V0 subunit d2 [KO:K02146]
2517  FUCA1; alpha-L-fucosidase 1 [KO:K01206] [EC:3.2.1.51]
2519  FUCA2; alpha-L-fucosidase 2 [KO:K01206] [EC:3.2.1.51]
2548  GAA; alpha glucosidase [KO:K12316] [EC:3.2.1.20]
256471  MFSD8; major facilitator superfamily domain containing 8 [KO:K12307]
2581  GALC; galactosylceramidase [KO:K01202] [EC:3.2.1.46]
2588  GALNS; galactosamine (N-acetyl)-6-sulfatase [KO:K01132] [EC:3.1.6.4]
26088  GGA1; golgi associated, gamma adaptin ear containing, ARF binding protein 1 [KO:K12404]
2629  GBA1; glucosylceramidase beta 1 [KO:K01201] [EC:3.2.1.45]
26503  SLC17A5; solute carrier family 17 member 5 [KO:K12301]
26985  AP3M1; adaptor related protein complex 3 subunit mu 1 [KO:K12398]
27074  LAMP3; lysosomal associated membrane protein 3 [KO:K06562]
2717  GLA; galactosidase alpha [KO:K01189] [EC:3.2.1.22]
2720  GLB1; galactosidase beta 1 [KO:K12309] [EC:3.2.1.23]
2760  GM2A; ganglioside GM2 activator [KO:K12383]
2799  GNS; glucosamine (N-acetyl)-6-sulfatase [KO:K01137] [EC:3.1.6.14]
285362  SUMF1; sulfatase modifying factor 1 [KO:K13444] [EC:1.8.3.7]
2990  GUSB; glucuronidase beta [KO:K01195] [EC:3.2.1.31]
3073  HEXA; hexosaminidase subunit alpha [KO:K12373] [EC:3.2.1.52]
3074  HEXB; hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
3373  HYAL1; hyaluronidase 1 [KO:K01197] [EC:3.2.1.35]
3423  IDS; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
3425  IDUA; alpha-L-iduronidase [KO:K01217] [EC:3.2.1.76]
3482  IGF2R; insulin like growth factor 2 receptor [KO:K06564]
3916  LAMP1; lysosomal associated membrane protein 1 [KO:K06528]
3920  LAMP2; lysosomal associated membrane protein 2 [KO:K06528]
3988  LIPA; lipase A, lysosomal acid type [KO:K01052] [EC:3.1.1.13]
4074  M6PR; mannose-6-phosphate receptor, cation dependent [KO:K10089]
410  ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
411  ARSB; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
4125  MAN2B1; mannosidase alpha class 2B member 1 [KO:K12311] [EC:3.2.1.24]
4126  MANBA; mannosidase beta [KO:K01192] [EC:3.2.1.25]
427  ASAH1; N-acylsphingosine amidohydrolase 1 [KO:K12348] [EC:3.5.1.23]
4668  NAGA; alpha-N-acetylgalactosaminidase [KO:K01204] [EC:3.2.1.49]
4669  NAGLU; N-acetyl-alpha-glucosaminidase [KO:K01205] [EC:3.2.1.50]
4758  NEU1; neuraminidase 1 [KO:K01186] [EC:3.2.1.18]
4864  NPC1; NPC intracellular cholesterol transporter 1 [KO:K12385]
4891  SLC11A2; solute carrier family 11 member 2 [KO:K21398]
50617  ATP6V0A4; ATPase H+ transporting V0 subunit a4 [KO:K02154]
51172  NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
51606  ATP6V1H; ATPase H+ transporting V1 subunit H [KO:K02144]
527  ATP6V0C; ATPase H+ transporting V0 subunit c [KO:K02155]
53  ACP2; acid phosphatase 2, lysosomal [KO:K14410] [EC:3.1.3.2]
533  ATP6V0B; ATPase H+ transporting V0 subunit b [KO:K03661]
535  ATP6V0A1; ATPase H+ transporting V0 subunit a1 [KO:K02154]
537  ATP6AP1; ATPase H+ transporting accessory protein 1 [KO:K03662]
54  ACP5; acid phosphatase 5, tartrate resistant [KO:K14379] [EC:3.1.3.2]
5476  CTSA; cathepsin A [KO:K13289] [EC:3.4.16.5]
55353  LAPTM4B; lysosomal protein transmembrane 4 beta [KO:K12387]
5538  PPT1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
5641  LGMN; legumain [KO:K01369] [EC:3.4.22.34]
5660  PSAP; prosaposin [KO:K12382]
57192  MCOLN1; mucolipin TRP cation channel 1 [KO:K04992]
57707  MEAK7; MTOR associated protein, eak-7 homolog [KO:K27947]
58511  DNASE2B; deoxyribonuclease 2 beta [KO:K01158] [EC:3.1.22.1]
6272  SORT1; sortilin 1 [KO:K12388]
6448  SGSH; N-sulfoglucosamine sulfohydrolase [KO:K01565] [EC:3.10.1.1]
6556  SLC11A1; solute carrier family 11 member 1 [KO:K12347]
6609  SMPD1; sphingomyelin phosphodiesterase 1 [KO:K12350] [EC:3.1.4.12]
6677  SPAM1; sperm adhesion molecule 1 [KO:K01197] [EC:3.2.1.35]
768239  PSAPL1; prosaposin like 1 [KO:K12382]
7805  LAPTM5; lysosomal protein transmembrane 5 [KO:K12387]
79158  GNPTAB; N-acetylglucosamine-1-phosphate transferase subunits alpha and beta [KO:K08239] [EC:2.7.8.17]
8120  AP3B2; adaptor related protein complex 3 subunit beta 2 [KO:K12397]
8218  CLTCL1; clathrin heavy chain like 1 [KO:K04646]
8372  HYAL3; hyaluronidase 3 [KO:K01197] [EC:3.2.1.35]
84572  GNPTG; N-acetylglucosamine-1-phosphate transferase subunit gamma [KO:K10087]
8546  AP3B1; adaptor related protein complex 3 subunit beta 1 [KO:K12397]
8692  HYAL2; hyaluronidase 2 [KO:K01197] [EC:3.2.1.35]
8722  CTSF; cathepsin F [KO:K01373] [EC:3.4.22.41]
8763  CD164; CD164 molecule [KO:K06546]
8905  AP1S2; adaptor related protein complex 1 subunit sigma 2 [KO:K12394]
8906  AP1G2; adaptor related protein complex 1 subunit gamma 2 [KO:K12391]
8907  AP1M1; adaptor related protein complex 1 subunit mu 1 [KO:K12393]
8943  AP3D1; adaptor related protein complex 3 subunit delta 1 [KO:K12396]
9114  ATP6V0D1; ATPase H+ transporting V0 subunit d1 [KO:K02146]
9179  AP4M1; adaptor related protein complex 4 subunit mu 1 [KO:K12402]
9374  PPT2; palmitoyl-protein thioesterase 2 [KO:K01074] [EC:3.1.2.22]
9476  NAPSA; napsin A aspartic peptidase [KO:K08565] [EC:3.4.23.-]
950  SCARB2; scavenger receptor class B member 2 [KO:K12384]
9516  LITAF; lipopolysaccharide induced TNF factor [KO:K19363]
9583  ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 [KO:K12305] [EC:3.6.1.6]
967  CD63; CD63 molecule [KO:K06497]
968  CD68; CD68 molecule [KO:K06501]
9741  LAPTM4A; lysosomal protein transmembrane 4 alpha [KO:K12387]
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
hsa00511  Other glycan degradation
hsa00531  Glycosaminoglycan degradation
hsa04130  SNARE interactions in vesicular transport
hsa04140  Autophagy - animal
hsa04144  Endocytosis
KO pathway
ko04142   

  All links  
No link information was found.
DBGET integrated database retrieval system