KEGG   PATHWAY: ko04142Help
Entry
ko04142                     Pathway                                

Name
Lysosome
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
ko04142

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
Other DBs
BSID: 
GO: 
Orthology
K02154  
V-type H+-transporting ATPase subunit a
K02146  
V-type H+-transporting ATPase subunit d
K02144  
V-type H+-transporting ATPase subunit H
K03662  
V-type H+-transporting ATPase S1 subunit
K02155  
V-type H+-transporting ATPase 16kDa proteolipid subunit
K03661  
V-type H+-transporting ATPase 21kDa proteolipid subunit
K13289  
cathepsin A (carboxypeptidase C) [EC:3.4.16.5]
K01363  
cathepsin B [EC:3.4.22.1]
K01275  
cathepsin C [EC:3.4.14.1]
K01379  
cathepsin D [EC:3.4.23.5]
K01382  
cathepsin E [EC:3.4.23.34]
K01373  
cathepsin F [EC:3.4.22.41]
K01319  
cathepsin G [EC:3.4.21.20]
K01366  
cathepsin H [EC:3.4.22.16]
K09599  
cathepsin P [EC:3.4.22.-]
K01371  
cathepsin K [EC:3.4.22.38]
K01365  
cathepsin L [EC:3.4.22.15]
K09600  
cathepsin M [EC:3.4.22.-]
K01374  
cathepsin O [EC:3.4.22.42]
K01368  
cathepsin S [EC:3.4.22.27]
K01375  
cathepsin V [EC:3.4.22.43]
K08569  
cathepsin W [EC:3.4.22.-]
K08568  
cathepsin X [EC:3.4.18.1]
K08565  
napsin-A [EC:3.4.23.-]
K01369  
legumain [EC:3.4.22.34]
K01279  
tripeptidyl-peptidase I [EC:3.4.14.9]
K01189  
alpha-galactosidase [EC:3.2.1.22]
K12309  
beta-galactosidase [EC:3.2.1.23]
K12316  
lysosomal alpha-glucosidase [EC:3.2.1.20]
K01201  
glucosylceramidase [EC:3.2.1.45]
K01217  
L-iduronidase [EC:3.2.1.76]
K01204  
alpha-N-acetylgalactosaminidase [EC:3.2.1.49]
K01205  
alpha-N-acetylglucosaminidase [EC:3.2.1.50]
K01202  
galactosylceramidase [EC:3.2.1.46]
K01195  
beta-glucuronidase [EC:3.2.1.31]
K12373  
hexosaminidase [EC:3.2.1.52]
K01192  
beta-mannosidase [EC:3.2.1.25]
K12311  
lysosomal alpha-mannosidase [EC:3.2.1.24]
K01186  
sialidase-1 [EC:3.2.1.18]
K01134  
arylsulfatase A [EC:3.1.6.8]
K01135  
arylsulfatase B [EC:3.1.6.12]
K12381  
arylsulfatase G [EC:3.1.6.-]
K01132  
N-acetylgalactosamine-6-sulfatase [EC:3.1.6.4]
K01137  
N-acetylglucosamine-6-sulfatase [EC:3.1.6.14]
K01136  
iduronate 2-sulfatase [EC:3.1.6.13]
K01565  
N-sulfoglucosamine sulfohydrolase [EC:3.10.1.1]
K01052  
lysosomal acid lipase/cholesteryl ester hydrolase [EC:3.1.1.13]
K06129  
lysophospholipase III [EC:3.1.1.5]
K01158  
deoxyribonuclease II [EC:3.1.22.1]
K12350  
sphingomyelin phosphodiesterase [EC:3.1.4.12]
K12348  
acid ceramidase [EC:3.5.1.23]
K01444  
N4-(beta-N-acetylglucosaminyl)-L-asparaginase [EC:3.5.1.26]
K12382  
saposin
K12383  
ganglioside GM2 activator
K01074  
palmitoyl-protein thioesterase [EC:3.1.2.22]
K06528  
lysosomal-associated membrane protein 1/2
K06562  
lysosomal-associated membrane protein 3
K06501  
CD68 antigen
K06497  
CD63 antigen
K12384  
lysosome membrane protein 2
K12385  
Niemann-Pick C1 protein
K13443  
Niemann-Pick C2 protein
K12386  
cystinosin
K12301  
MFS transporter, ACS family, solute carrier family 17 (sodium-dependent inorganic phosphate cotransporter), member 5
K12347  
natural resistance-associated macrophage protein
K12387  
lysosomal-associated transmembrane protein
K05642  
ATP-binding cassette, subfamily A (ABC1), member 2
K05656  
ATP-binding cassette, subfamily B (MDR/TAP), member 9
K06546  
CD164 antigen
K12305  
ectonucleoside triphosphate diphosphohydrolase 4 [EC:3.6.1.6]
K12388  
sortilin
K12389  
battenin
K12390  
ceroid-lipofuscinosis neuronal protein 5
K12307  
MFS transporter, ceroid-lipofuscinosis neuronal protein 7
K10532  
heparan-alpha-glucosaminide N-acetyltransferase [EC:2.3.1.78]
K13444  
sulfatase modifying factor 1
K08239  
UDP-N-acetylglucosamine-lysosomal-enzyme [EC:2.7.8.17]
K10087  
N-acetylglucosamine-1-phosphate transferase, gamma subunit
K01125  
N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase [EC:3.1.4.45]
K06564  
insulin-like growth factor 2 receptor
K10089  
cation-dependent mannose-6-phosphate receptor
K04644  
clathrin light chain A
K04645  
clathrin light chain B
K04646  
clathrin heavy chain
K12391  
AP-1 complex subunit gamma-1
K12392  
AP-1 complex subunit beta-1
K12393  
AP-1 complex subunit mu
K12394  
AP-1 complex subunit sigma 1/2
K12395  
AP-1 complex subunit sigma-3
K12396  
AP-3 complex subunit delta-1
K12397  
AP-3 complex subunit beta
K12398  
AP-3 complex subunit mu
K12399  
AP-3 complex subunit sigma
K12400  
AP-4 complex subunit epsilon-1
K12401  
AP-4 complex subunit beta-1
K12402  
AP-4 complex subunit mu-1
K12403  
AP-4 complex subunit sigma-1
K12404  
ADP-ribosylation factor-binding protein GGA
K04992  
mucolipin 1
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)

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