KEGG   PATHWAY: hgl04142
Entry
hgl04142                    Pathway                                
Name
Lysosome - Heterocephalus glaber (naked mole-rat)
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
hgl04142  Lysosome
hgl04142

Other DBs
GO: 0005764
Organism
Heterocephalus glaber (naked mole-rat) [GN:hgl]
Gene
101700877  Atp6v0a2; ATPase H+ transporting V0 subunit a2 [KO:K02154]
101719254  Atp6v0a4; ATPase H+ transporting V0 subunit a4 [KO:K02154]
101720677  Tcirg1; T-cell immune regulator 1, ATPase H+ transporting V0 subunit a3 [KO:K02154]
101718747  Atp6v0a1; ATPase H+ transporting V0 subunit a1 [KO:K02154]
101711869  Atp6v0d1; ATPase H+ transporting V0 subunit d1 [KO:K02146]
101696829  Atp6v0d2; ATPase H+ transporting V0 subunit d2 [KO:K02146]
101710987  Atp6v1h; ATPase H+ transporting V1 subunit H [KO:K02144]
101705200  Atp6ap1; ATPase H+ transporting accessory protein 1 [KO:K03662]
101700782  V-type proton ATPase 16 kDa proteolipid subunit-like [KO:K02155]
101709243  Atp6v0c; ATPase H+ transporting V0 subunit c [KO:K02155]
101718458  Atp6v0b; ATPase H+ transporting V0 subunit b [KO:K03661]
101715839  Dmxl1; Dmx like 1 [KO:K24155]
101705947  Dmxl2; Dmx like 2 [KO:K24155]
101711846  Wdr7; WD repeat domain 7 [KO:K24738]
101716476  Ncoa7; nuclear receptor coactivator 7 [KO:K25442]
101720484  Ctsa; cathepsin A [KO:K13289] [EC:3.4.16.5]
101709008  Ctsb; cathepsin B [KO:K01363] [EC:3.4.22.1]
101697606  Ctsc; cathepsin C [KO:K01275] [EC:3.4.14.1]
101716979  Ctsd; cathepsin D [KO:K01379] [EC:3.4.23.5]
101699894  Ctse; cathepsin E [KO:K01382] [EC:3.4.23.34]
101719972  Ctsf; cathepsin F [KO:K01373] [EC:3.4.22.41]
101713070  cathepsin G [KO:K01319] [EC:3.4.21.20]
101712232  cathepsin G [KO:K01319] [EC:3.4.21.20]
101700582  Ctsh; cathepsin H [KO:K01366] [EC:3.4.22.16]
101716993  Ctsk; cathepsin K [KO:K01371] [EC:3.4.22.38]
101717733  cathepsin L1-like [KO:K01365] [EC:3.4.22.15]
101724540  Ctso; cathepsin O [KO:K01374] [EC:3.4.22.42]
101697008  Ctss; cathepsin S [KO:K01368] [EC:3.4.22.27]
101708790  Ctsw; cathepsin W [KO:K08569] [EC:3.4.22.-]
101720244  Ctsz; cathepsin Z [KO:K08568] [EC:3.4.18.1]
101706612  Napsa; napsin A aspartic peptidase [KO:K08565] [EC:3.4.23.-]
101698288  Lgmn; legumain [KO:K01369] [EC:3.4.22.34]
101707185  Tpp1; tripeptidyl peptidase 1 [KO:K01279] [EC:3.4.14.9]
101713601  Gla; galactosidase alpha [KO:K01189] [EC:3.2.1.22]
101712908  Glb1; galactosidase beta 1 [KO:K12309] [EC:3.2.1.23]
101718861  Gaa; glucosidase alpha, acid [KO:K12316] [EC:3.2.1.20]
101704109  Gba; glucosylceramidase beta [KO:K01201] [EC:3.2.1.45]
101725783  Idua; iduronidase, alpha-L- [KO:K01217] [EC:3.2.1.76]
101726122  Naga; alpha-N-acetylgalactosaminidase [KO:K01204] [EC:3.2.1.49]
101723414  Naglu; N-acetyl-alpha-glucosaminidase [KO:K01205] [EC:3.2.1.50]
101721890  Galc; galactosylceramidase [KO:K01202] [EC:3.2.1.46]
101709573  Gusb; glucuronidase beta [KO:K01195] [EC:3.2.1.31]
101723569  Fuca2; fucosidase, alpha-L- 2, plasma [KO:K01206] [EC:3.2.1.51]
101717431  Fuca1; fucosidase, alpha-L- 1, tissue [KO:K01206] [EC:3.2.1.51]
101713145  Hexb; hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
101718368  Hexa; hexosaminidase subunit alpha [KO:K12373] [EC:3.2.1.52]
101712953  Manba; mannosidase beta [KO:K01192] [EC:3.2.1.25]
101705792  Man2b1; mannosidase alpha class 2B member 1 [KO:K12311] [EC:3.2.1.24]
101699194  Neu1; neuraminidase 1 [KO:K01186] [EC:3.2.1.18]
101701149  Hyal1; hyaluronoglucosaminidase 1 [KO:K01197] [EC:3.2.1.35]
101701738  Hyal3; hyaluronoglucosaminidase 3 [KO:K01197] [EC:3.2.1.35]
101709759  Hyal2; hyaluronoglucosaminidase 2 [KO:K01197] [EC:3.2.1.35]
101723286  Hyal4; hyaluronoglucosaminidase 4 [KO:K01197] [EC:3.2.1.35]
101723647  hyaluronidase-4-like [KO:K01197] [EC:3.2.1.35]
101700218  Spam1; sperm adhesion molecule 1 [KO:K01197] [EC:3.2.1.35]
101722754  Arsa; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
101719731  Arsb; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
101720783  Arsg; arylsulfatase G [KO:K12381] [EC:3.1.6.-]
101712119  Galns; galactosamine (N-acetyl)-6-sulfatase [KO:K01132] [EC:3.1.6.4]
101710393  Gns; glucosamine (N-acetyl)-6-sulfatase [KO:K01137] [EC:3.1.6.14]
101710577  Ids; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
101720611  Sgsh; N-sulfoglucosamine sulfohydrolase [KO:K01565] [EC:3.10.1.1]
101713991  Lipa; lipase A, lysosomal acid type [KO:K01052] [EC:3.1.1.13]
101698711  Pla2g15; phospholipase A2 group XV [KO:K06129] [EC:3.1.1.5]
101719320  Dnase2b; deoxyribonuclease 2 beta [KO:K01158] [EC:3.1.22.1]
101716162  Dnase2; deoxyribonuclease 2, lysosomal [KO:K01158] [EC:3.1.22.1]
101715936  Acp2; acid phosphatase 2, lysosomal [KO:K14410] [EC:3.1.3.2]
101718093  Acp5; acid phosphatase 5, tartrate resistant [KO:K14379] [EC:3.1.3.2]
101699079  Smpd1; sphingomyelin phosphodiesterase 1 [KO:K12350] [EC:3.1.4.12]
101724913  Asah1; N-acylsphingosine amidohydrolase 1 [KO:K12348] [EC:3.5.1.23]
101721618  Aga; aspartylglucosaminidase [KO:K01444] [EC:3.5.1.26]
101722989  Psap; prosaposin [KO:K12382]
106009578  Psapl1; prosaposin-like 1 [KO:K12382]
101706300  Gm2a; GM2 ganglioside activator [KO:K12383]
101713926  Ppt2; palmitoyl-protein thioesterase 2 [KO:K01074] [EC:3.1.2.22]
101718692  Ppt1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
101710608  Lamp2; lysosomal associated membrane protein 2 [KO:K06528]
101702697  Lamp1; lysosomal associated membrane protein 1 [KO:K06528]
101725065  Lamp3; lysosomal associated membrane protein 3 [KO:K06562]
101698291  Cd68; CD68 molecule [KO:K06501]
101699645  Cd63; CD63 molecule [KO:K06497]
101715798  Scarb2; scavenger receptor class B member 2 [KO:K12384]
101710344  Npc1; NPC intracellular cholesterol transporter 1 [KO:K12385]
101700822  Npc2; NPC intracellular cholesterol transporter 2 [KO:K13443]
101721174  Ctns; cystinosin, lysosomal cystine transporter [KO:K12386]
101720632  Slc17a5; solute carrier family 17 member 5 [KO:K12301]
101707446  Slc11a1; solute carrier family 11 member 1 [KO:K12347]
101723788  Slc11a2; solute carrier family 11 member 2 [KO:K21398]
101723178  Laptm4a; lysosomal protein transmembrane 4 alpha [KO:K12387]
101711304  Laptm4b; lysosomal protein transmembrane 4 beta [KO:K12387]
101697987  Laptm5; lysosomal protein transmembrane 5 [KO:K12387]
101697102  Abca2; ATP binding cassette subfamily A member 2 [KO:K05642]
101716118  Abcb9; ATP binding cassette subfamily B member 9 [KO:K05656]
101701393  Cd164; CD164 molecule [KO:K06546]
101700645  Entpd4; ectonucleoside triphosphate diphosphohydrolase 4 [KO:K12305] [EC:3.6.1.6]
101698000  Sort1; sortilin 1 [KO:K12388]
101722930  Cln3; CLN3, battenin [KO:K12389]
101715781  Cln5; ceroid-lipofuscinosis, neuronal 5 [KO:K12390]
101699855  Mfsd8; major facilitator superfamily domain containing 8 [KO:K12307]
101698026  Hgsnat; heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
101704938  Sumf1; sulfatase modifying factor 1 [KO:K13444] [EC:1.8.3.7]
101710539  Gnptab; N-acetylglucosamine-1-phosphate transferase alpha and beta subunits [KO:K08239] [EC:2.7.8.17]
101715733  Gnptg; N-acetylglucosamine-1-phosphate transferase gamma subunit [KO:K10087]
101704527  Nagpa; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
101707470  Igf2r; insulin like growth factor 2 receptor [KO:K06564]
101704683  M6pr; mannose-6-phosphate receptor, cation dependent [KO:K10089]
101707717  Clta; clathrin light chain A [KO:K04644]
101713269  Cltb; clathrin light chain B [KO:K04645]
101708690  Cltcl1; clathrin heavy chain like 1 [KO:K04646]
101720552  Cltc; clathrin heavy chain [KO:K04646]
101700868  Ap1g1; adaptor related protein complex 1 gamma 1 subunit [KO:K12391]
101708508  Ap1g2; adaptor related protein complex 1 gamma 2 subunit [KO:K12391]
101702149  Ap1b1; adaptor related protein complex 1 beta 1 subunit [KO:K12392]
101705051  Ap1m1; adaptor related protein complex 1 mu 1 subunit [KO:K12393]
101721337  Ap1m2; adaptor related protein complex 1 mu 2 subunit [KO:K12393]
101703538  Ap1s1; adaptor related protein complex 1 sigma 1 subunit [KO:K12394]
101721754  AP-1 complex subunit sigma-2 [KO:K12394]
101697909  AP-1 complex subunit sigma-2 [KO:K12394]
101717662  AP-1 complex subunit sigma-2 [KO:K12394]
101702055  Ap1s2; adaptor related protein complex 1 sigma 2 subunit [KO:K12394]
101698822  Ap1s3; adaptor related protein complex 1 sigma 3 subunit [KO:K12395]
101713323  Ap3d1; adaptor related protein complex 3 delta 1 subunit [KO:K12396]
101701517  Ap3b2; adaptor related protein complex 3 beta 2 subunit [KO:K12397]
101701726  Ap3b1; adaptor related protein complex 3 beta 1 subunit [KO:K12397]
101718782  Ap3m1; adaptor related protein complex 3 mu 1 subunit [KO:K12398]
101712189  Ap3m2; adaptor related protein complex 3 mu 2 subunit [KO:K12398]
101720828  Ap3s1; adaptor related protein complex 3 sigma 1 subunit [KO:K12399]
101707009  Ap3s2; adaptor related protein complex 3 sigma 2 subunit [KO:K12399]
101716970  AP-3 complex subunit sigma-1-like [KO:K12399]
101708335  Ap4e1; adaptor related protein complex 4 epsilon 1 subunit [KO:K12400]
106007798  AP-4 complex subunit epsilon-1-like [KO:K12400]
101714886  Ap4b1; adaptor related protein complex 4 beta 1 subunit [KO:K12401]
101725856  Ap4m1; adaptor related protein complex 4 mu 1 subunit [KO:K12402]
101699663  Ap4s1; adaptor related protein complex 4 sigma 1 subunit [KO:K12403]
101720393  Gga1; golgi associated, gamma adaptin ear containing, ARF binding protein 1 [KO:K12404]
101718247  Gga2; golgi associated, gamma adaptin ear containing, ARF binding protein 2 [KO:K12404]
101713542  Gga3; golgi associated, gamma adaptin ear containing, ARF binding protein 3 [KO:K12404]
101697714  Mcoln1; mucolipin 1 [KO:K04992]
101725320  Litaf; lipopolysaccharide induced TNF factor [KO:K19363]
Compound
C00002  ATP
C00008  ADP
C00159  D-Mannose
C00275  D-Mannose 6-phosphate
Reference
  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
  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
  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
  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
  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
hgl00511  Other glycan degradation
hgl00531  Glycosaminoglycan degradation
hgl04130  SNARE interactions in vesicular transport
hgl04140  Autophagy - animal
hgl04144  Endocytosis
KO pathway
ko04142   
LinkDB

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