KEGG PATHWAY: ko04142

PATHWAY: ko04142

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

Pathway map

ko04142

Lysosome

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 type C disease (NPC)
H00137	Niemann-Pick disease (NPD), including;
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
H00696	Haim-Munk syndrome
H00810	Progressive myoclonic epilepsy (PME)
H00932	Tropical calcific pancreatitis
H01097	Cerebral palsy
H01113	Acid phosphatase deficiency
H01196	Hypochromic microcytic anemia
H01239	Combined SAP deficiency

Other DBs

BSID:

96865

GO:

0005764

Orthology

K02154	V-type H+-transporting ATPase subunit I [EC:3.6.3.14]
K02146	V-type H+-transporting ATPase subunit AC39 [EC:3.6.3.14]
K02144	V-type H+-transporting ATPase 54 kD subunit [EC:3.6.3.14]
K03662	V-type H+-transporting ATPase S1 subunit [EC:3.6.3.14]
K02155	V-type H+-transporting ATPase 16kDa proteolipid subunit [EC:3.6.3.14]
K03661	V-type H+-transporting ATPase 21kDa proteolipid subunit [EC:3.6.3.14]
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

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)

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

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)

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)

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Ontology (2)   
   KEGG BRITE (1)   
   GO (1)   
Pathway (2)   
   KEGG PATHWAY (1)   
   BioSystems (1)   
Disease (41)   
   KEGG DISEASE (41)   
Chemical substance (4)   
   KEGG COMPOUND (4)   
Chemical reaction (50)   
   KEGG ENZYME (50)   
Gene (99)   
   KEGG ORTHOLOGY (99)   
Literature (5)   
   PubMed (5)   
All databases (203)   

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