KEGG   PATHWAY: hsa04142Help
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
BRITE hierarchy
Pathway map
Lysosome
hsa04142

All organismsOrtholog table
Disease
H00123  
Mucopolysaccharidosis type IV (MPS4)
H00124  
GM2 gangliosidoses
H00125  
Fabry disease
H00126  
Gaucher disease
H00127  
Metachromatic leukodystrophy (MLD)
H00128  
Mucopolysaccharidosis type I (MPS1)
H00129  
Mucopolysaccharidosis type II (MPS2)
H00130  
Mucopolysaccharidosis type III (MPS3)
H00131  
Mucopolysaccharidosis type VI (MPS6)
H00132  
Mucopolysaccharidosis type VII (MPS7)
H00133  
Mucopolysaccharidosis type IX (MPS9)
H00135  
Krabbe disease
H00136  
Niemann-Pick disease type C (NPC)
H00137  
Niemann-Pick disease (NPD) typeA and B
H00138  
Farber lipogranulomatosis
H00139  
alpha-Mannosidosis
H00140  
beta-Mannosidosis
H00141  
Fucosidosis
H00142  
Sialidosis
H00143  
Mucolipidosis II and III
H00144  
Mucolipidosis IV
H00145  
Aspartylglucosaminuria (AGU)
H00146  
Alpha-N-acetylgalactosaminidase deficiency
H00147  
Sialuria/ Sialic acid storage disease
H00148  
Lysosomal acid lipase deficiency
H00149  
Neuronal ceroid lipofuscinosis
H00150  
Danon disease
H00166  
Hermansky-Pudlak syndrome (HPS)
H00272  
Multiple sulfatase deficiency (MSD)
H00273  
Pycnodysostosis
H00274  
Papillon-Lefevre syndrome
H00275  
Cystinosis
H00276  
Galactosialidosis
H00281  
GM1 gangliosidosis
H00421  
Mucopolysaccharidosis (MPS)
H00422  
Glycoproteinoses
H00423  
Defects in the degradation of sulfatide
H00424  
Defects in the degradation of sphingomyelin
H00425  
Lysosomal cysteine protease deficiencies
H00426  
Defects in the degradation of ganglioside
H00696  
Haim-Munk syndrome
H00810  
Progressive myoclonic epilepsy (PME/ EPM)
H00932  
Tropical calcific pancreatitis
H01097  
Cerebral palsy
H01113  
Acid phosphatase deficiency
H01196  
Hypochromic microcytic anemia
H01239  
Combined SAP deficiency
Drug
D06634  
Relacatib (USAN/INN)
D08955  
Odanacatib (JAN/USAN)
Other DBs
BSID: 
GO: 
Organism
Homo sapiens (human) [GN:hsa]
Gene
10312  
TCIRG1; T-cell, immune regulator 1, ATPase, H+ transporting, lysosomal V0 subunit A3 [KO:K02154]
23545  
ATP6V0A2; ATPase, H+ transporting, lysosomal V0 subunit a2 [KO:K02154]
50617  
ATP6V0A4; ATPase, H+ transporting, lysosomal V0 subunit a4 [KO:K02154]
535  
ATP6V0A1; ATPase, H+ transporting, lysosomal V0 subunit a1 [KO:K02154]
9114  
ATP6V0D1; ATPase, H+ transporting, lysosomal 38kDa, V0 subunit d1 [KO:K02146]
245972  
ATP6V0D2; ATPase, H+ transporting, lysosomal 38kDa, V0 subunit d2 [KO:K02146]
51606  
ATP6V1H; ATPase, H+ transporting, lysosomal 50/57kDa, V1 subunit H [KO:K02144]
537  
ATP6AP1; ATPase, H+ transporting, lysosomal accessory protein 1 [KO:K03662]
527  
ATP6V0C; ATPase, H+ transporting, lysosomal 16kDa, V0 subunit c [KO:K02155]
533  
ATP6V0B; ATPase, H+ transporting, lysosomal 21kDa, V0 subunit b [KO:K03661]
5476  
CTSA; cathepsin A [KO:K13289] [EC:3.4.16.5]
1508  
CTSB; cathepsin B [KO:K01363] [EC:3.4.22.1]
1075  
CTSC; cathepsin C [KO:K01275] [EC:3.4.14.1]
1509  
CTSD; cathepsin D [KO:K01379] [EC:3.4.23.5]
1510  
CTSE; cathepsin E [KO:K01382] [EC:3.4.23.34]
8722  
CTSF; cathepsin F [KO:K01373] [EC:3.4.22.41]
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]
1519  
CTSO; cathepsin O [KO:K01374] [EC:3.4.22.42]
1520  
CTSS; cathepsin S [KO:K01368] [EC:3.4.22.27]
1515  
CTSV; cathepsin V [KO:K01375] [EC:3.4.22.43]
1521  
CTSW; cathepsin W [KO:K08569] [EC:3.4.22.-]
1522  
CTSZ; cathepsin Z [KO:K08568] [EC:3.4.18.1]
9476  
NAPSA; napsin A aspartic peptidase [KO:K08565] [EC:3.4.23.-]
5641  
LGMN; legumain [KO:K01369] [EC:3.4.22.34]
1200  
TPP1; tripeptidyl peptidase I [KO:K01279] [EC:3.4.14.9]
2717  
GLA; galactosidase, alpha [KO:K01189] [EC:3.2.1.22]
2720  
GLB1; galactosidase, beta 1 [KO:K12309] [EC:3.2.1.23]
2548  
GAA; glucosidase, alpha [KO:K12316] [EC:3.2.1.20]
2629  
GBA; glucosidase, beta, acid [KO:K01201] [EC:3.2.1.45]
3425  
IDUA; iduronidase, alpha-L- [KO:K01217] [EC:3.2.1.76]
4668  
NAGA; N-acetylgalactosaminidase, alpha- [KO:K01204] [EC:3.2.1.49]
4669  
NAGLU; N-acetylglucosaminidase, alpha [KO:K01205] [EC:3.2.1.50]
2581  
GALC; galactosylceramidase [KO:K01202] [EC:3.2.1.46]
2990  
GUSB; glucuronidase, beta [KO:K01195] [EC:3.2.1.31]
3073  
HEXA; hexosaminidase A (alpha polypeptide) [KO:K12373] [EC:3.2.1.52]
3074  
HEXB; hexosaminidase B (beta polypeptide) [KO:K12373] [EC:3.2.1.52]
4126  
MANBA; mannosidase, beta A, lysosomal [KO:K01192] [EC:3.2.1.25]
4125  
MAN2B1; mannosidase, alpha, class 2B, member 1 [KO:K12311] [EC:3.2.1.24]
4758  
NEU1; sialidase 1 (lysosomal sialidase) [KO:K01186] [EC:3.2.1.18]
410  
ARSA; arylsulfatase A [KO:K01134] [EC:3.1.6.8]
411  
ARSB; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
22901  
ARSG; arylsulfatase G [KO:K12381] [EC:3.1.6.-]
2588  
GALNS; galactosamine (N-acetyl)-6-sulfate sulfatase [KO:K01132] [EC:3.1.6.4]
2799  
GNS; glucosamine (N-acetyl)-6-sulfatase [KO:K01137] [EC:3.1.6.14]
3423  
IDS; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
6448  
SGSH; N-sulfoglucosamine sulfohydrolase [KO:K01565] [EC:3.10.1.1]
3988  
LIPA; lipase A, lysosomal acid, cholesterol esterase [KO:K01052] [EC:3.1.1.13]
23659  
PLA2G15; phospholipase A2, group XV [KO:K06129] [EC:3.1.1.5]
1777  
DNASE2; deoxyribonuclease II, lysosomal [KO:K01158] [EC:3.1.22.1]
58511  
DNASE2B; deoxyribonuclease II beta [KO:K01158] [EC:3.1.22.1]
6609  
SMPD1; sphingomyelin phosphodiesterase 1, acid lysosomal [KO:K12350] [EC:3.1.4.12]
427  
ASAH1; N-acylsphingosine amidohydrolase (acid ceramidase) 1 [KO:K12348] [EC:3.5.1.23]
175  
AGA; aspartylglucosaminidase [KO:K01444] [EC:3.5.1.26]
5660  
PSAP; prosaposin [KO:K12382]
768239  
PSAPL1; prosaposin-like 1 (gene/pseudogene) [KO:K12382]
2760  
GM2A; GM2 ganglioside activator [KO:K12383]
5538  
PPT1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
9374  
PPT2; palmitoyl-protein thioesterase 2 [KO:K01074] [EC:3.1.2.22]
3916  
LAMP1; lysosomal-associated membrane protein 1 [KO:K06528]
3920  
LAMP2; lysosomal-associated membrane protein 2 [KO:K06528]
27074  
LAMP3; lysosomal-associated membrane protein 3 [KO:K06562]
968  
CD68; CD68 molecule [KO:K06501]
967  
CD63; CD63 molecule [KO:K06497]
950  
SCARB2; scavenger receptor class B, member 2 [KO:K12384]
4864  
NPC1; Niemann-Pick disease, type C1 [KO:K12385]
10577  
NPC2; Niemann-Pick disease, type C2 [KO:K13443]
1497  
CTNS; cystinosin, lysosomal cystine transporter [KO:K12386]
26503  
SLC17A5; solute carrier family 17 (acidic sugar transporter), member 5 [KO:K12301]
6556  
SLC11A1; solute carrier family 11 (proton-coupled divalent metal ion transporter), member 1 [KO:K12347]
4891  
SLC11A2; solute carrier family 11 (proton-coupled divalent metal ion transporter), member 2 [KO:K12347]
55353  
LAPTM4B; lysosomal protein transmembrane 4 beta [KO:K12387]
7805  
LAPTM5; lysosomal protein transmembrane 5 [KO:K12387]
9741  
LAPTM4A; lysosomal protein transmembrane 4 alpha [KO:K12387]
20  
ABCA2; ATP-binding cassette, sub-family A (ABC1), member 2 [KO:K05642]
23457  
ABCB9; ATP-binding cassette, sub-family B (MDR/TAP), member 9 [KO:K05656]
8763  
CD164; CD164 molecule, sialomucin [KO:K06546]
9583  
ENTPD4; ectonucleoside triphosphate diphosphohydrolase 4 [KO:K12305] [EC:3.6.1.6]
6272  
SORT1; sortilin 1 [KO:K12388]
1201  
CLN3; ceroid-lipofuscinosis, neuronal 3 [KO:K12389]
1203  
CLN5; ceroid-lipofuscinosis, neuronal 5 [KO:K12390]
256471  
MFSD8; major facilitator superfamily domain containing 8 [KO:K12307]
138050  
HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
285362  
SUMF1; sulfatase modifying factor 1 [KO:K13444]
79158  
GNPTAB; N-acetylglucosamine-1-phosphate transferase, alpha and beta subunits [KO:K08239] [EC:2.7.8.17]
84572  
GNPTG; N-acetylglucosamine-1-phosphate transferase, gamma subunit [KO:K10087]
51172  
NAGPA; N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [KO:K01125] [EC:3.1.4.45]
3482  
IGF2R; insulin-like growth factor 2 receptor [KO:K06564]
4074  
M6PR; mannose-6-phosphate receptor (cation dependent) [KO:K10089]
1211  
CLTA; clathrin, light chain A [KO:K04644]
1212  
CLTB; clathrin, light chain B [KO:K04645]
1213  
CLTC; clathrin, heavy chain (Hc) [KO:K04646]
8218  
CLTCL1; clathrin, heavy chain-like 1 [KO:K04646]
164  
AP1G1; adaptor-related protein complex 1, gamma 1 subunit [KO:K12391]
8906  
AP1G2; adaptor-related protein complex 1, gamma 2 subunit [KO:K12391]
162  
AP1B1; adaptor-related protein complex 1, beta 1 subunit [KO:K12392]
8907  
AP1M1; adaptor-related protein complex 1, mu 1 subunit [KO:K12393]
10053  
AP1M2; adaptor-related protein complex 1, mu 2 subunit [KO:K12393]
1174  
AP1S1; adaptor-related protein complex 1, sigma 1 subunit [KO:K12394]
8905  
AP1S2; adaptor-related protein complex 1, sigma 2 subunit [KO:K12394]
130340  
AP1S3; adaptor-related protein complex 1, sigma 3 subunit [KO:K12395]
8943  
AP3D1; adaptor-related protein complex 3, delta 1 subunit [KO:K12396]
8120  
AP3B2; adaptor-related protein complex 3, beta 2 subunit [KO:K12397]
8546  
AP3B1; adaptor-related protein complex 3, beta 1 subunit [KO:K12397]
26985  
AP3M1; adaptor-related protein complex 3, mu 1 subunit [KO:K12398]
10947  
AP3M2; adaptor-related protein complex 3, mu 2 subunit [KO:K12398]
10239  
AP3S2; adaptor-related protein complex 3, sigma 2 subunit [KO:K12399]
1176  
AP3S1; adaptor-related protein complex 3, sigma 1 subunit [KO:K12399]
23431  
AP4E1; adaptor-related protein complex 4, epsilon 1 subunit [KO:K12400]
10717  
AP4B1; adaptor-related protein complex 4, beta 1 subunit [KO:K12401]
9179  
AP4M1; adaptor-related protein complex 4, mu 1 subunit [KO:K12402]
11154  
AP4S1; adaptor-related protein complex 4, sigma 1 subunit [KO:K12403]
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]
26088  
GGA1; golgi-associated, gamma adaptin ear containing, ARF binding protein 1 [KO:K12404]
57192  
MCOLN1; mucolipin 1 [KO:K04992]
2517  
FUCA1; fucosidase, alpha-L- 1, tissue [KO:K01206] [EC:3.2.1.51]
3373  
HYAL1; hyaluronoglucosaminidase 1 [KO:K01197] [EC:3.2.1.35]
53  
ACP2; acid phosphatase 2, lysosomal [KO:K14410] [EC:3.1.3.2]
54  
ACP5; acid phosphatase 5, tartrate resistant [KO:K14379] [EC:3.1.3.2]
Compound
C00002  
ATP
C00008  
ADP
C00159  
D-Mannose
C00275  
D-Mannose 6-phosphate
Reference
  Authors
Alberts B,  Johnson A, Lewis J, Raff M, Roberts K, Walter P
  Title
Molecular Biology of the Cell 4th ed.
  Journal
Garland Science (2002)
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)
Reference
PMID:1883197
  Authors
Neufeld EF
  Title
Lysosomal storage diseases.
  Journal
Annu Rev Biochem 60:257-80 (1991)
Reference
PMID:2943218
  Authors
von Figura K, Hasilik A
  Title
Lysosomal enzymes and their receptors.
  Journal
Annu Rev Biochem 55:167-93 (1986)
Reference
  Authors
Uchiyama Y.
  Title
[The analysis of a knockout mouse.] ( In Japanese)
  Journal
Cell Technology 25:1322-27 (2006)
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)
Reference
  Authors
Vergarajauregui S, Puertollano R
  Title
Mucolipidosis type IV: the importance of functional lysosomes for efficient autophagy.
  Journal
Autophagy 4:832-4 (2008)
KO pathway
 

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