KEGG   PATHWAY: mlx04142
Entry
mlx04142                    Pathway                                
Name
Lysosome - Mirounga leonina (Southern elephant seal)
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
mlx04142  Lysosome
mlx04142

Other DBs
GO: 0005764
Organism
Mirounga leonina (Southern elephant seal) [GN:mlx]
Gene
118016148  TCIRG1; V-type proton ATPase 116 kDa subunit a isoform X1 [KO:K02154]
118024916  ATP6V0A1; V-type proton ATPase 116 kDa subunit a isoform X1 [KO:K02154]
118018379  ATP6V0A2; V-type proton ATPase 116 kDa subunit a isoform X1 [KO:K02154]
118021138  ATP6V0A4; V-type proton ATPase 116 kDa subunit a [KO:K02154]
118014982  ATP6V0D2; V-type proton ATPase subunit d 2 [KO:K02146]
118023671  ATP6V0D1; V-type proton ATPase subunit d 1 [KO:K02146]
118014820  ATP6V1H; V-type proton ATPase subunit H isoform X1 [KO:K02144]
118014535  ATP6AP1; V-type proton ATPase subunit S1 [KO:K03662]
118021734  ATP6V0C; V-type proton ATPase 16 kDa proteolipid subunit [KO:K02155]
118019515  ATP6V0B; V-type proton ATPase 21 kDa proteolipid subunit [KO:K03661]
118016557  DMXL1; dmX-like protein 1 isoform X1 [KO:K24155]
118003720  DMXL2; dmX-like protein 2 isoform X1 [KO:K24155]
118004399  WDR7; WD repeat-containing protein 7 isoform X1 [KO:K24738]
118000697  NCOA7; nuclear receptor coactivator 7 isoform X1 [KO:K25442]
118011546  CTSA; lysosomal protective protein [KO:K13289] [EC:3.4.16.5]
118022144  CTSB; cathepsin B [KO:K01363] [EC:3.4.22.1]
118013461  CTSC; dipeptidyl peptidase 1 isoform X1 [KO:K01275] [EC:3.4.14.1]
118016198  CTSD; cathepsin D [KO:K01379] [EC:3.4.23.5]
118013666  CTSE; cathepsin E isoform X1 [KO:K01382] [EC:3.4.23.34]
118016101  CTSF; LOW QUALITY PROTEIN: cathepsin F [KO:K01373] [EC:3.4.22.41]
118003492  CTSG; cathepsin G [KO:K01319] [EC:3.4.21.20]
118023131  CTSH; pro-cathepsin H [KO:K01366] [EC:3.4.22.16]
118000314  CTSK; cathepsin K [KO:K01371] [EC:3.4.22.38]
118023332  LOW QUALITY PROTEIN: cathepsin L1-like [KO:K01365] [EC:3.4.22.15]
118023333  cathepsin L1-like [KO:K01365] [EC:3.4.22.15]
118023348  cathepsin L1 [KO:K01365] [EC:3.4.22.15]
118022211  CTSO; cathepsin O isoform X1 [KO:K01374] [EC:3.4.22.42]
118000310  CTSS; cathepsin S isoform X1 [KO:K01368] [EC:3.4.22.27]
118016071  CTSW; cathepsin W [KO:K08569] [EC:3.4.22.-]
118024013  CTSZ; cathepsin Z [KO:K08568] [EC:3.4.18.1]
117998301  NAPSA; napsin-A [KO:K08565] [EC:3.4.23.-]
118002628  LGMN; legumain isoform X1 [KO:K01369] [EC:3.4.22.34]
118004087  TPP1; tripeptidyl-peptidase 1 [KO:K01279] [EC:3.4.14.9]
118022775  GLA; alpha-galactosidase A [KO:K01189] [EC:3.2.1.22]
118001485  GLB1; beta-galactosidase [KO:K12309] [EC:3.2.1.23]
118024195  GAA; lysosomal alpha-glucosidase [KO:K12316] [EC:3.2.1.20]
118013879  GBA; lysosomal acid glucosylceramidase [KO:K01201] [EC:3.2.1.45]
118010810  IDUA; alpha-L-iduronidase isoform X1 [KO:K01217] [EC:3.2.1.76]
117998614  NAGA; alpha-N-acetylgalactosaminidase isoform X1 [KO:K01204] [EC:3.2.1.49]
118024917  NAGLU; alpha-N-acetylglucosaminidase isoform X1 [KO:K01205] [EC:3.2.1.50]
118002521  GALC; galactocerebrosidase isoform X1 [KO:K01202] [EC:3.2.1.46]
117999537  GUSB; beta-glucuronidase isoform X1 [KO:K01195] [EC:3.2.1.31]
118000950  FUCA2; plasma alpha-L-fucosidase isoform X1 [KO:K01206] [EC:3.2.1.51]
118019883  FUCA1; tissue alpha-L-fucosidase isoform X1 [KO:K01206] [EC:3.2.1.51]
118017134  HEXB; beta-hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
118003676  HEXA; beta-hexosaminidase subunit alpha isoform X1 [KO:K12373] [EC:3.2.1.52]
118027048  MANBA; beta-mannosidase isoform X1 [KO:K01192] [EC:3.2.1.25]
118005098  MAN2B1; lysosomal alpha-mannosidase isoform X1 [KO:K12311] [EC:3.2.1.24]
118027320  NEU1; sialidase-1 isoform X1 [KO:K01186] [EC:3.2.1.18]
118005108  hyaluronidase-1 isoform X1 [KO:K01197] [EC:3.2.1.35]
118005305  HYAL3; hyaluronidase-3 isoform X1 [KO:K01197] [EC:3.2.1.35]
118021385  HYAL4; hyaluronidase-4 [KO:K01197] [EC:3.2.1.35]
118021386  SPAM1; hyaluronidase PH-20 [KO:K01197] [EC:3.2.1.35]
117997725  ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
118016931  ARSB; arylsulfatase B isoform X1 [KO:K01135] [EC:3.1.6.12]
118024336  ARSG; arylsulfatase G isoform X1 [KO:K12381] [EC:3.1.6.-]
118023463  GALNS; N-acetylgalactosamine-6-sulfatase isoform X1 [KO:K01132] [EC:3.1.6.4]
118008620  GNS; N-acetylglucosamine-6-sulfatase [KO:K01137] [EC:3.1.6.14]
118008400  IDS; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
118024193  SGSH; N-sulphoglucosamine sulphohydrolase isoform X1 [KO:K01565] [EC:3.10.1.1]
118012956  LIPA; lysosomal acid lipase/cholesteryl ester hydrolase isoform X1 [KO:K01052] [EC:3.1.1.13]
118023643  PLA2G15; phospholipase A2 group XV isoform X1 [KO:K06129] [EC:3.1.1.5]
118005335  DNASE2; LOW QUALITY PROTEIN: deoxyribonuclease-2-alpha [KO:K01158] [EC:3.1.22.1]
118020190  DNASE2B; deoxyribonuclease-2-beta [KO:K01158] [EC:3.1.22.1]
118015831  ACP2; lysosomal acid phosphatase [KO:K14410] [EC:3.1.3.2]
118005349  ACP5; tartrate-resistant acid phosphatase type 5 [KO:K14379] [EC:3.1.3.2]
118004084  SMPD1; sphingomyelin phosphodiesterase isoform X1 [KO:K12350] [EC:3.1.4.12]
118010040  ASAH1; acid ceramidase isoform X1 [KO:K12348] [EC:3.5.1.23]
118010091  AGA; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase [KO:K01444] [EC:3.5.1.26]
118021088  PSAP; prosaposin isoform X1 [KO:K12382]
118006680  uncharacterized protein LOC118006680 isoform X1 [KO:K12383]
118006823  ganglioside GM2 activator-like [KO:K12383]
118027306  PPT2; lysosomal thioesterase PPT2 [KO:K01074] [EC:3.1.2.22]
118019578  PPT1; palmitoyl-protein thioesterase 1 isoform X1 [KO:K01074] [EC:3.1.2.22]
118009217  LAMP1; lysosome-associated membrane glycoprotein 1 isoform X1 [KO:K06528]
118012464  LAMP2; lysosome-associated membrane glycoprotein 2 isoform X1 [KO:K06528]
118006869  LAMP3; lysosome-associated membrane glycoprotein 3 isoform X1 [KO:K06562]
118000028  CD68; macrosialin [KO:K06501]
118008745  CD63; CD63 antigen [KO:K06497]
118011455  CD63 antigen-like [KO:K06497]
118010430  SCARB2; lysosome membrane protein 2 [KO:K12384]
118004529  NPC1; NPC intracellular cholesterol transporter 1 [KO:K12385]
118002040  NPC2; NPC intracellular cholesterol transporter 2 [KO:K13443]
118025173  CTNS; cystinosin [KO:K12386]
118000567  SLC17A5; sialin isoform X1 [KO:K12301]
118012047  SLC11A1; natural resistance-associated macrophage protein 1 isoform X1 [KO:K12347]
118008847  SLC11A2; natural resistance-associated macrophage protein 2 [KO:K21398]
118007624  LAPTM4A; lysosomal-associated transmembrane protein 4A [KO:K12387]
118015050  LAPTM4B; lysosomal-associated transmembrane protein 4B [KO:K12387]
118019779  LAPTM5; lysosomal-associated transmembrane protein 5 [KO:K12387]
118017499  ABCA2; ATP-binding cassette sub-family A member 2 isoform X1 [KO:K05642]
118018407  ABCB9; ATP-binding cassette sub-family B member 9 isoform X1 [KO:K05656]
118001585  CD164; sialomucin core protein 24 [KO:K06546]
118022092  ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 isoform X1 [KO:K12305] [EC:3.6.1.6]
118019036  SORT1; sortilin isoform X1 [KO:K12388]
117999652  CLN3; battenin [KO:K12389]
118009323  CLN5; ceroid-lipofuscinosis neuronal protein 5 [KO:K12390]
118022336  MFSD8; major facilitator superfamily domain-containing protein 8 [KO:K12307]
118009905  HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase isoform X1 [KO:K10532] [EC:2.3.1.78]
118005936  SUMF1; formylglycine-generating enzyme isoform X1 [KO:K13444] [EC:1.8.3.7]
117998822  GNPTAB; N-acetylglucosamine-1-phosphotransferase subunits alpha/beta isoform X1 [KO:K08239] [EC:2.7.8.17]
118021688  GNPTG; N-acetylglucosamine-1-phosphotransferase subunit gamma [KO:K10087]
118021805  NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
118001837  cation-independent mannose-6-phosphate receptor [KO:K06564]
118001960  cation-independent mannose-6-phosphate receptor [KO:K06564]
118025693  M6PR; cation-dependent mannose-6-phosphate receptor isoform X1 [KO:K10089]
118017949  CLTA; clathrin light chain A isoform X1 [KO:K04644]
118016980  CLTB; clathrin light chain B [KO:K04645]
118024684  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
117999766  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
118023599  AP1G1; AP-1 complex subunit gamma-1 isoform X1 [KO:K12391]
118003157  AP1G2; AP-1 complex subunit gamma-like 2 isoform X1 [KO:K12391]
118018628  AP1B1; AP-1 complex subunit beta-1 isoform X1 [KO:K12392]
118005100  AP1M2; AP-1 complex subunit mu-2 isoform X1 [KO:K12393]
118006097  AP1M1; AP-1 complex subunit mu-1 [KO:K12393]
117999440  AP1S1; AP-1 complex subunit sigma-1A [KO:K12394]
118007046  AP1S2; AP-1 complex subunit sigma-2 isoform X1 [KO:K12394]
118012635  AP1S3; AP-1 complex subunit sigma-3 [KO:K12395]
118006040  AP3D1; AP-3 complex subunit delta-1 isoform X1 [KO:K12396]
118023097  AP3B2; AP-3 complex subunit beta-2 isoform X1 [KO:K12397]
118016926  AP3B1; AP-3 complex subunit beta-1 isoform X1 [KO:K12397]
118009925  AP3M2; AP-3 complex subunit mu-2 [KO:K12398]
118021808  AP3M1; AP-3 complex subunit mu-1 isoform X1 [KO:K12398]
118023057  AP-3 complex subunit sigma-2 isoform X1 [KO:K12399]
118016546  AP3S1; AP-3 complex subunit sigma-1 isoform X1 [KO:K12399]
118003609  AP4E1; AP-4 complex subunit epsilon-1 isoform X1 [KO:K12400]
118000506  AP4B1; AP-4 complex subunit beta-1 isoform X1 [KO:K12401]
117999395  AP4M1; AP-4 complex subunit mu-1 isoform X1 [KO:K12402]
118003077  AP4S1; AP-4 complex subunit sigma-1 [KO:K12403]
118024274  GGA3; ADP-ribosylation factor-binding protein GGA3 [KO:K12404]
117998706  GGA1; ADP-ribosylation factor-binding protein GGA1 isoform X1 [KO:K12404]
117999129  GGA2; ADP-ribosylation factor-binding protein GGA2 isoform X1 [KO:K12404]
118005979  MCOLN1; mucolipin-1 isoform X1 [KO:K04992]
118021833  LITAF; lipopolysaccharide-induced tumor necrosis factor-alpha 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
mlx00511  Other glycan degradation
mlx00531  Glycosaminoglycan degradation
mlx04130  SNARE interactions in vesicular transport
mlx04140  Autophagy - animal
mlx04144  Endocytosis
KO pathway
ko04142   
LinkDB

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