KEGG   PATHWAY: mmu04142
Entry
mmu04142                    Pathway                                
Name
Lysosome - Mus musculus (house mouse)
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
mmu04142  Lysosome
mmu04142

Other DBs
GO: 0005764
Organism
Mus musculus (house mouse) [GN:mmu]
Gene
11975  Atp6v0a1; ATPase, H+ transporting, lysosomal V0 subunit A1 [KO:K02154]
21871  Atp6v0a2; ATPase, H+ transporting, lysosomal V0 subunit A2 [KO:K02154]
27060  Tcirg1; T cell, immune regulator 1, ATPase, H+ transporting, lysosomal V0 protein A3 [KO:K02154]
140494  Atp6v0a4; ATPase, H+ transporting, lysosomal V0 subunit A4 [KO:K02154]
11972  Atp6v0d1; ATPase, H+ transporting, lysosomal V0 subunit D1 [KO:K02146]
242341  Atp6v0d2; ATPase, H+ transporting, lysosomal V0 subunit D2 [KO:K02146]
108664  Atp6v1h; ATPase, H+ transporting, lysosomal V1 subunit H [KO:K02144]
54411  Atp6ap1; ATPase, H+ transporting, lysosomal accessory protein 1 [KO:K03662]
11984  Atp6v0c; ATPase, H+ transporting, lysosomal V0 subunit C [KO:K02155]
114143  Atp6v0b; ATPase, H+ transporting, lysosomal V0 subunit B [KO:K03661]
240283  Dmxl1; Dmx-like 1 [KO:K24155]
235380  Dmxl2; Dmx-like 2 [KO:K24155]
104082  Wdr7; WD repeat domain 7 [KO:K24738]
211329  Ncoa7; nuclear receptor coactivator 7 [KO:K25442]
19025  Ctsa; cathepsin A [KO:K13289] [EC:3.4.16.5]
13030  Ctsb; cathepsin B [KO:K01363] [EC:3.4.22.1]
13032  Ctsc; cathepsin C [KO:K01275] [EC:3.4.14.1]
13033  Ctsd; cathepsin D [KO:K01379] [EC:3.4.23.5]
13034  Ctse; cathepsin E [KO:K01382] [EC:3.4.23.34]
56464  Ctsf; cathepsin F [KO:K01373] [EC:3.4.22.41]
13035  Ctsg; cathepsin G [KO:K01319] [EC:3.4.21.20]
13036  Ctsh; cathepsin H [KO:K01366] [EC:3.4.22.16]
26898  Ctsj; cathepsin J [KO:K09599] [EC:3.4.22.-]
13038  Ctsk; cathepsin K [KO:K01371] [EC:3.4.22.38]
13039  Ctsl; cathepsin L [KO:K01365] [EC:3.4.22.15]
64139  Ctsm; cathepsin M [KO:K09600] [EC:3.4.22.-]
229445  Ctso; cathepsin O [KO:K01374] [EC:3.4.22.42]
13040  Ctss; cathepsin S [KO:K01368] [EC:3.4.22.27]
13041  Ctsw; cathepsin W [KO:K08569] [EC:3.4.22.-]
64138  Ctsz; cathepsin Z [KO:K08568] [EC:3.4.18.1]
16541  Napsa; napsin A aspartic peptidase [KO:K08565] [EC:3.4.23.-]
19141  Lgmn; legumain [KO:K01369] [EC:3.4.22.34]
12751  Tpp1; tripeptidyl peptidase I [KO:K01279] [EC:3.4.14.9]
11605  Gla; galactosidase, alpha [KO:K01189] [EC:3.2.1.22]
12091  Glb1; galactosidase, beta 1 [KO:K12309] [EC:3.2.1.23]
14387  Gaa; glucosidase, alpha, acid [KO:K12316] [EC:3.2.1.20]
14466  Gba1; glucosylceramidase beta 1 [KO:K01201] [EC:3.2.1.45]
15932  Idua; iduronidase, alpha-L [KO:K01217] [EC:3.2.1.76]
17939  Naga; N-acetyl galactosaminidase, alpha [KO:K01204] [EC:3.2.1.49]
27419  Naglu; alpha-N-acetylglucosaminidase (Sanfilippo disease IIIB) [KO:K01205] [EC:3.2.1.50]
14420  Galc; galactosylceramidase [KO:K01202] [EC:3.2.1.46]
110006  Gusb; glucuronidase, beta [KO:K01195] [EC:3.2.1.31]
71665  Fuca1; fucosidase, alpha-L- 1, tissue [KO:K01206] [EC:3.2.1.51]
66848  Fuca2; fucosidase, alpha-L- 2, plasma [KO:K01206] [EC:3.2.1.51]
15211  Hexa; hexosaminidase A [KO:K12373] [EC:3.2.1.52]
15212  Hexb; hexosaminidase B [KO:K12373] [EC:3.2.1.52]
110173  Manba; mannosidase, beta A, lysosomal [KO:K01192] [EC:3.2.1.25]
17159  Man2b1; mannosidase 2, alpha B1 [KO:K12311] [EC:3.2.1.24]
18010  Neu1; neuraminidase 1 [KO:K01186] [EC:3.2.1.18]
20690  Spam1; sperm adhesion molecule 1 [KO:K01197] [EC:3.2.1.35]
15586  Hyal1; hyaluronoglucosaminidase 1 [KO:K01197] [EC:3.2.1.35]
15587  Hyal2; hyaluronoglucosaminidase 2 [KO:K01197] [EC:3.2.1.35]
74468  Hyal5; hyaluronoglucosaminidase 5 [KO:K01197] [EC:3.2.1.35]
77042  Hyal4; hyaluronoglucosaminidase 4 [KO:K01197] [EC:3.2.1.35]
109685  Hyal3; hyaluronoglucosaminidase 3 [KO:K01197] [EC:3.2.1.35]
74409  Hyal6; hyaluronoglucosaminidase 6 [KO:K01197] [EC:3.2.1.35]
11883  Arsa; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
11881  Arsb; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
74008  Arsg; arylsulfatase G [KO:K12381] [EC:3.1.6.-]
50917  Galns; galactosamine (N-acetyl)-6-sulfatase [KO:K01132] [EC:3.1.6.4]
75612  Gns; glucosamine (N-acetyl)-6-sulfatase [KO:K01137] [EC:3.1.6.14]
15931  Ids; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
27029  Sgsh; N-sulfoglucosamine sulfohydrolase (sulfamidase) [KO:K01565] [EC:3.10.1.1]
16889  Lipa; lysosomal acid lipase A [KO:K01052] [EC:3.1.1.13]
192654  Pla2g15; phospholipase A2, group XV [KO:K06129] [EC:3.1.1.5]
13423  Dnase2a; deoxyribonuclease II alpha [KO:K01158] [EC:3.1.22.1]
56629  Dnase2b; deoxyribonuclease II beta [KO:K01158] [EC:3.1.22.1]
11432  Acp2; acid phosphatase 2, lysosomal [KO:K14410] [EC:3.1.3.2]
11433  Acp5; acid phosphatase 5, tartrate resistant [KO:K14379] [EC:3.1.3.2]
20597  Smpd1; sphingomyelin phosphodiesterase 1, acid lysosomal [KO:K12350] [EC:3.1.4.12]
11886  Asah1; N-acylsphingosine amidohydrolase 1 [KO:K12348] [EC:3.5.1.23]
11593  Aga; aspartylglucosaminidase [KO:K01444] [EC:3.5.1.26]
19156  Psap; prosaposin [KO:K12382]
76943  Psapl1; prosaposin-like 1 [KO:K12382]
14667  Gm2a; GM2 ganglioside activator protein [KO:K12383]
19063  Ppt1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
54397  Ppt2; palmitoyl-protein thioesterase 2 [KO:K01074] [EC:3.1.2.22]
16783  Lamp1; lysosomal-associated membrane protein 1 [KO:K06528]
16784  Lamp2; lysosomal-associated membrane protein 2 [KO:K06528]
239739  Lamp3; lysosomal-associated membrane protein 3 [KO:K06562]
12514  Cd68; CD68 antigen [KO:K06501]
12512  Cd63; CD63 antigen [KO:K06497]
12492  Scarb2; scavenger receptor class B, member 2 [KO:K12384]
18145  Npc1; NPC intracellular cholesterol transporter 1 [KO:K12385]
67963  Npc2; NPC intracellular cholesterol transporter 2 [KO:K13443]
83429  Ctns; cystinosis, nephropathic [KO:K12386]
235504  Slc17a5; solute carrier family 17 (anion/sugar transporter), member 5 [KO:K12301]
18173  Slc11a1; solute carrier family 11 (proton-coupled divalent metal ion transporters), member 1 [KO:K12347]
18174  Slc11a2; solute carrier family 11 (proton-coupled divalent metal ion transporters), member 2 [KO:K21398]
114128  Laptm4b; lysosomal-associated protein transmembrane 4B [KO:K12387]
17775  Laptm4a; lysosomal-associated protein transmembrane 4A [KO:K12387]
16792  Laptm5; lysosomal-associated protein transmembrane 5 [KO:K12387]
11305  Abca2; ATP-binding cassette, sub-family A member 2 [KO:K05642]
56325  Abcb9; ATP-binding cassette, sub-family B member 9 [KO:K05656]
53599  Cd164; CD164 antigen [KO:K06546]
67464  Entpd4; ectonucleoside triphosphate diphosphohydrolase 4 [KO:K12305] [EC:3.6.1.6]
100862375  Entpd4b; ectonucleoside triphosphate diphosphohydrolase 4B [KO:K12305] [EC:3.6.1.6]
20661  Sort1; sortilin 1 [KO:K12388]
12752  Cln3; CLN3 lysosomal/endosomal transmembrane protein, battenin [KO:K12389]
211286  Cln5; ceroid-lipofuscinosis, neuronal 5 [KO:K12390]
72175  Mfsd8; major facilitator superfamily domain containing 8 [KO:K12307]
52120  Hgsnat; heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
58911  Sumf1; sulfatase modifying factor 1 [KO:K13444] [EC:1.8.3.7]
432486  Gnptab; N-acetylglucosamine-1-phosphate transferase, alpha and beta subunits [KO:K08239] [EC:2.7.8.17]
214505  Gnptg; N-acetylglucosamine-1-phosphotransferase, gamma subunit [KO:K10087]
27426  Nagpa; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
16004  Igf2r; insulin-like growth factor 2 receptor [KO:K06564]
17113  M6pr; mannose-6-phosphate receptor, cation dependent [KO:K10089]
12757  Clta; clathrin light chain A [KO:K04644]
74325  Cltb; clathrin light chain B [KO:K04645]
67300  Cltc; clathrin heavy chain [KO:K04646]
11765  Ap1g1; adaptor protein complex AP-1, gamma 1 subunit [KO:K12391]
11766  Ap1g2; adaptor protein complex AP-1, gamma 2 subunit [KO:K12391]
11764  Ap1b1; adaptor protein complex AP-1, beta 1 subunit [KO:K12392]
11767  Ap1m1; adaptor-related protein complex AP-1, mu subunit 1 [KO:K12393]
11768  Ap1m2; adaptor protein complex AP-1, mu 2 subunit [KO:K12393]
108012  Ap1s2; adaptor-related protein complex 1, sigma 2 subunit [KO:K12394]
11769  Ap1s1; adaptor protein complex AP-1, sigma 1 [KO:K12394]
252903  Ap1s3; adaptor-related protein complex AP-1, sigma 3 [KO:K12395]
11776  Ap3d1; adaptor-related protein complex 3, delta 1 subunit [KO:K12396]
11774  Ap3b1; adaptor-related protein complex 3, beta 1 subunit [KO:K12397]
11775  Ap3b2; adaptor-related protein complex 3, beta 2 subunit [KO:K12397]
55946  Ap3m1; adaptor-related protein complex 3, mu 1 subunit [KO:K12398]
64933  Ap3m2; adaptor-related protein complex 3, mu 2 subunit [KO:K12398]
11778  Ap3s2; adaptor-related protein complex 3, sigma 2 subunit [KO:K12399]
11777  Ap3s1; adaptor-related protein complex 3, sigma 1 subunit [KO:K12399]
108011  Ap4e1; adaptor-related protein complex AP-4, epsilon 1 [KO:K12400]
67489  Ap4b1; adaptor-related protein complex AP-4, beta 1 [KO:K12401]
11781  Ap4m1; adaptor-related protein complex AP-4, mu 1 [KO:K12402]
11782  Ap4s1; adaptor-related protein complex AP-4, sigma 1 [KO:K12403]
106039  Gga1; golgi associated, gamma adaptin ear containing, ARF binding protein 1 [KO:K12404]
260302  Gga3; golgi associated, gamma adaptin ear containing, ARF binding protein 3 [KO:K12404]
74105  Gga2; golgi associated, gamma adaptin ear containing, ARF binding protein 2 [KO:K12404]
94178  Mcoln1; mucolipin 1 [KO:K04992]
56722  Litaf; LPS-induced TN 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
mmu00511  Other glycan degradation
mmu00531  Glycosaminoglycan degradation
mmu04130  SNARE interactions in vesicular transport
mmu04140  Autophagy - animal
mmu04144  Endocytosis
KO pathway
ko04142   
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