KEGG   PATHWAY: hhip04142
Entry
hhip04142                   Pathway                                
Name
Lysosome - Hippoglossus hippoglossus (Atlantic halibut)
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
hhip04142  Lysosome
hhip04142

Other DBs
GO: 0005764
Organism
Hippoglossus hippoglossus (Atlantic halibut) [GN:hhip]
Gene
117752119  mfsd8; major facilitator superfamily domain-containing protein 8 [KO:K12307]
117752128  [KO:K02146]
117752449  lipf; gastric triacylglycerol lipase [KO:K01052] [EC:3.1.1.13]
117752473  arsg; arylsulfatase G [KO:K12381] [EC:3.1.6.-]
117752502  [KO:K02154]
117752593  atp6v0cb; ATPase H+ transporting V0 subunit cb [KO:K02155]
117753020  naglu; alpha-N-acetylglucosaminidase [KO:K01205] [EC:3.2.1.50]
117753331  lamp1a; lysosome-associated membrane glycoprotein 1a [KO:K06528]
117753373  gnptg; N-acetylglucosamine-1-phosphotransferase subunit gamma [KO:K10087]
117753376  si:ch211-122f10.4; lysosomal protective protein [KO:K13289] [EC:3.4.16.5]
117753422  [KO:K19363]
117753631  acp5b; tartrate-resistant acid phosphatase type 5b [KO:K14379] [EC:3.1.3.2]
117754074  ap3s2; AP-3 complex subunit sigma-2 [KO:K12399]
117754118  dmxl2; dmX-like protein 2 isoform X1 [KO:K24155]
117754538  ctsh; pro-cathepsin H isoform X1 [KO:K01366] [EC:3.4.22.16]
117754574  man2b1; lysosomal alpha-mannosidase [KO:K12311] [EC:3.2.1.24]
117755247  cln5; ceroid-lipofuscinosis neuronal protein 5 [KO:K12390]
117755368  lamp1b; lysosome-associated membrane glycoprotein 1b [KO:K06528]
117755564  fuca2; plasma alpha-L-fucosidase [KO:K01206] [EC:3.2.1.51]
117755842  si:ch211-15d5.11; nuclear receptor coactivator 7 isoform X1 [KO:K25442]
117756166  ap4s1; AP-4 complex subunit sigma-1 isoform X1 [KO:K12403]
117756331  manba; beta-mannosidase [KO:K01192] [EC:3.2.1.25]
117756363  tpp1; tripeptidyl-peptidase 1 isoform X1 [KO:K01279] [EC:3.4.14.9]
117756410  [KO:K13443]
117756532  ctsba; cathepsin B [KO:K01363] [EC:3.4.22.1]
117756559  galcb; galactocerebrosidase isoform X1 [KO:K01202] [EC:3.2.1.46]
117756672  lgmn; legumain [KO:K01369] [EC:3.4.22.34]
117757039  nots; nothepsin [KO:K01382] [EC:3.4.23.34]
117757181  gnptab; N-acetylglucosamine-1-phosphotransferase subunits alpha/beta isoform X1 [KO:K08239] [EC:2.7.8.17]
117757456  si:dkey-159a18.1; sortilin [KO:K12388]
117757514  [KO:K01197] [EC:3.2.1.35]
117757535  gnsa; N-acetylglucosamine-6-sulfatase [KO:K01137] [EC:3.1.6.14]
117757973  laptm4a; lysosomal-associated transmembrane protein 4A [KO:K12387]
117757991  [KO:K12394]
117758051  igf2r; cation-independent mannose-6-phosphate receptor isoform X1 [KO:K06564]
117758079  [KO:K25442]
117758130  cd164; sialomucin core protein 24 isoform X1 [KO:K06546]
117758411  [KO:K01363] [EC:3.4.22.1]
117758879  naga; alpha-N-acetylgalactosaminidase [KO:K01204] [EC:3.2.1.49]
117759144  galns; N-acetylgalactosamine-6-sulfatase [KO:K01132] [EC:3.1.6.4]
117759155  ctsd; cathepsin D isoform X1 [KO:K01379] [EC:3.4.23.5]
117759545  ap1g1; AP-1 complex subunit gamma-1 isoform X1 [KO:K12391]
117759716  [KO:K12394]
117759921  [KO:K01158] [EC:3.1.22.1]
117759922  [KO:K01158] [EC:3.1.22.1]
117759926  [KO:K01158] [EC:3.1.22.1]
117759927  [KO:K01158] [EC:3.1.22.1]
117760047  ap3d1; AP-3 complex subunit delta-1 isoform X1 [KO:K12396]
117760319  ap1m3; adaptor related protein complex 1 subunit mu 3 [KO:K12393]
117760385  mfsd8l1; LOW QUALITY PROTEIN: uncharacterized protein mfsd8l1 [KO:K12307]
117760435  cts12; cathepsin L1 [KO:K01365] [EC:3.4.22.15]
117760509  [KO:K06562]
117760772  ctns; cystinosin [KO:K12386]
117760819  si:ch73-12o23.1; LOW QUALITY PROTEIN: lysosomal alpha-glucosidase [KO:K12316] [EC:3.2.1.20]
117761138  hyal2b; hyaluronidase-2 [KO:K01197] [EC:3.2.1.35]
117761194  ctsz; cathepsin Z [KO:K08568] [EC:3.4.18.1]
117761196  [KO:K21398]
117761491  [KO:K12388]
117761537  hyal2a; hyaluronidase-2 [KO:K01197] [EC:3.2.1.35]
117761538  hyal1; hyaluronidase-1 [KO:K01197] [EC:3.2.1.35]
117761887  sumf1; formylglycine-generating enzyme [KO:K13444] [EC:1.8.3.7]
117762170  ctsa; lysosomal protective protein [KO:K13289] [EC:3.4.16.5]
117762295  mcoln1a; mucolipin-1a isoform X1 [KO:K04992]
117762556  hgsnat; heparan-alpha-glucosaminide N-acetyltransferase isoform X1 [KO:K10532] [EC:2.3.1.78]
117762591  ap4e1; AP-4 complex subunit epsilon-1 isoform X1 [KO:K12400]
117763086  hyal6; hyaluronoglucosaminidase 6 isoform X1 [KO:K01197] [EC:3.2.1.35]
117763160  arsa; arylsulfatase A isoform X1 [KO:K01134] [EC:3.1.6.8]
117763423  pla2g15; group XV phospholipase A2 [KO:K06129] [EC:3.1.1.5]
117763491  [KO:K01197] [EC:3.2.1.35]
117763637  [KO:K01197] [EC:3.2.1.35]
117763650  [KO:K12397]
117763722  [KO:K01197] [EC:3.2.1.35]
117764188  hyal3; hyaluronidase-3 [KO:K01197] [EC:3.2.1.35]
117764217  [KO:K03662]
117764245  [KO:K03662]
117764299  [KO:K19363]
117764321  sort1b; sortilin 1b [KO:K12388]
117764356  [KO:K03662]
117764477  cd63; CD63 antigen [KO:K06497]
117764619  [KO:K21398]
117764707  si:dkey-72l14.3; glyco_hydro_56 domain-containing protein [KO:K01197] [EC:3.2.1.35]
117765187  [KO:K12404]
117765387  ap4b1; AP-4 complex subunit beta-1 isoform X1 [KO:K12401]
117765913  atp6v0a1a; V-type proton ATPase 116 kDa subunit a isoform X1 [KO:K02154]
117766124  [KO:K19363]
117766177  glb1; beta-galactosidase [KO:K12309] [EC:3.2.1.23]
117766237  [KO:K12404]
117766388  [KO:K19363]
117766469  mcoln1b; mucolipin-1 [KO:K04992]
117766568  napsa; napsin-A [KO:K01379] [EC:3.4.23.5]
117766593  gaa; lysosomal alpha-glucosidase isoform X1 [KO:K12316] [EC:3.2.1.20]
117766762  ap1m2; AP-1 complex subunit mu-2 [KO:K12393]
117766778  atp6v0ca; ATPase H+ transporting V0 subunit ca [KO:K02155]
117766894  cln3; battenin isoform X1 [KO:K12389]
117766937  gga3a; ADP-ribosylation factor-binding protein GGA3a isoform X1 [KO:K12404]
117766978  gga3b; ADP-ribosylation factor-binding protein GGA3 [KO:K12404]
117767438  ap1b1; AP-1 complex subunit beta-1 isoform X1 [KO:K12392]
117767471  [KO:K12399]
117767503  [KO:K24155]
117767571  scarb2a; lysosome membrane protein 2a isoform X1 [KO:K12384]
117767579  cltcl1; clathrin heavy chain 1 isoform X1 [KO:K04646]
117767804  abca2; ATP-binding cassette sub-family A member 2 isoform X1 [KO:K05642]
117768074  idua; alpha-L-iduronidase [KO:K01217] [EC:3.2.1.76]
117768105  ctsla; cathepsin La [KO:K01365] [EC:3.4.22.15]
117768163  ap3m2; AP-3 complex subunit mu-2 [KO:K12398]
117768242  [KO:K02154]
117768356  [KO:K12404]
117768451  entpd4; ectonucleoside triphosphate diphosphohydrolase 4 isoform X1 [KO:K12305] [EC:3.6.1.6]
117768502  sftpbb; surfactant protein Bb [KO:K12382]
117768568  abcb9; ATP-binding cassette sub-family B member 9 [KO:K05656]
117768791  gla; alpha-galactosidase A [KO:K01189] [EC:3.2.1.22]
117768808  sgsh; N-sulphoglucosamine sulphohydrolase [KO:K01565] [EC:3.10.1.1]
117768821  gm2a; ganglioside GM2 activator [KO:K12383]
117769171  lamp2; lysosome-associated membrane glycoprotein 2 isoform X1 [KO:K06528]
117769232  ids; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
117769239  [KO:K08568] [EC:3.4.18.1]
117769298  cltb; clathrin light chain B isoform X1 [KO:K04645]
117769497  aga; N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase [KO:K01444] [EC:3.5.1.26]
117769537  ctso; cathepsin O isoform X1 [KO:K01374] [EC:3.4.22.42]
117769849  [KO:K02154]
117769859  clta; clathrin light chain A isoform X1 [KO:K04644]
117769871  ctsf; cathepsin F [KO:K01373] [EC:3.4.22.41]
117770089  neu1; sialidase-1 [KO:K01186] [EC:3.2.1.18]
117770104  si:dkeyp-75b4.8; lipopolysaccharide-induced tumor necrosis factor-alpha factor homolog [KO:K19363]
117770115  ppt2b; lysosomal thioesterase PPT2 [KO:K01074] [EC:3.1.2.22]
117770265  [KO:K01206] [EC:3.2.1.51]
117770319  [KO:K01368] [EC:3.4.22.27]
117770320  [KO:K01371] [EC:3.4.22.38]
117770321  [KO:K01371] [EC:3.4.22.38]
117770342  laptm4b; lysosomal-associated transmembrane protein 4B [KO:K12387]
117770427  [KO:K01206] [EC:3.2.1.51]
117770435  asah1b; N-acylsphingosine amidohydrolase (acid ceramidase) 1b isoform X1 [KO:K12348] [EC:3.5.1.23]
117770567  ppt1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
117770947  si:ch1073-513e17.1; sialin [KO:K12301]
117771295  [KO:K24738]
117771311  acp5a; tartrate-resistant acid phosphatase type 5a [KO:K14379] [EC:3.1.3.2]
117771464  hexb; beta-hexosaminidase subunit beta isoform X1 [KO:K12373] [EC:3.2.1.52]
117771690  arsb; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
117771757  atp6v0a2a; V-type proton ATPase 116 kDa subunit a [KO:K02154]
117771767  [KO:K12382]
117771794  ap3b1a; AP-3 complex subunit beta-1 isoform X1 [KO:K12397]
117771819  scarb2c; lysosome membrane protein 2c [KO:K12384]
117771838  [KO:K12384]
117771856  [KO:K19363]
117771909  gnsb; glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), b isoform X1 [KO:K01137] [EC:3.1.6.14]
117772718  ap1s3a; AP-1 complex subunit sigma-3a [KO:K12395]
117773206  ctsc; dipeptidyl peptidase 1 [KO:K01275] [EC:3.4.14.1]
117773308  acp2; lysosomal acid phosphatase isoform X1 [KO:K14410] [EC:3.1.3.2]
117773465  [KO:K04646]
117774032  gusb; beta-glucuronidase [KO:K01195] [EC:3.2.1.31]
117774084  cltca; clathrin, heavy chain a (Hc) isoform X1 [KO:K04646]
117774375  [KO:K12350] [EC:3.1.4.12]
117774686  ap4m1; AP-4 complex subunit mu-1 isoform X1 [KO:K12402]
117775273  slc17a5; sialin isoform X1 [KO:K12301]
117775327  si:dkey-192p21.6; heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
117775988  ap3m1; AP-3 complex subunit mu-1 [KO:K12398]
117776075  psap; prosaposin isoform X1 [KO:K12382]
117776271  dnase2; deoxyribonuclease-2-alpha isoform X1 [KO:K01158] [EC:3.1.22.1]
117776373  nagpa; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
117776444  ctss2.1; cathepsin S, ortholog2, tandem duplicate 1 isoform X1 [KO:K01368] [EC:3.4.22.27]
117776445  ctsk; cathepsin K [KO:K01371] [EC:3.4.22.38]
117776449  [KO:K12387]
117776619  gba; lysosomal acid glucosylceramidase isoform X1 [KO:K01201] [EC:3.2.1.45]
117776822  m6pr; cation-dependent mannose-6-phosphate receptor isoform X1 [KO:K10089]
117777054  [KO:K01074] [EC:3.1.2.22]
117777657  [KO:K08568] [EC:3.4.18.1]
117777711  npc1; NPC intracellular cholesterol transporter 1 [KO:K12385]
117777736  ap1m1; AP-1 complex subunit mu-1 [KO:K12393]
117778143  [KO:K02144]
117778209  mfsd8l2; major facilitator superfamily domain-containing protein 8 [KO:K12307]
117778394  atp6v0b; V-type proton ATPase 21 kDa proteolipid subunit [KO:K03661]
117778475  [KO:K02146]
117778514  ap1s3b; AP-1 complex subunit sigma-3b isoform X1 [KO:K12394]
117778909  ap1g2; AP-1 complex subunit gamma-like 2 [KO:K12391]
117779180  si:ch211-212k18.7; macrosialin isoform X1 [KO:K06501]
117779323  ap1s1; AP-1 complex subunit sigma-1A [KO:K12394]
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
hhip00511  Other glycan degradation
hhip00531  Glycosaminoglycan degradation
hhip04130  SNARE interactions in vesicular transport
hhip04140  Autophagy - animal
hhip04144  Endocytosis
KO pathway
ko04142   

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