KEGG PATHWAY: ko04120

PATHWAY: ko04120

Entry

ko04120 Pathway

Name

Ubiquitin mediated proteolysis

Description

Protein ubiquitination plays an important role in eukaryotic cellular processes. It mainly functions as a signal for 26S proteasome dependent protein degradation. The addition of ubiquitin to proteins being degraded is performed by a reaction cascade consisting of three enzymes, named E1 (ubiquitin activating enzyme), E2 (ubiquitin conjugating enzyme), and E3 (ubiquitin ligase). Each E3 has specificity to its substrate, or proteins to be targeted by ubiquitination. Many E3s are discovered in eukaryotes and they are classified into four types: HECT type, U-box type, single RING-finger type, and multi-subunit RING-finger type. Multi-subunit RING-finger E3s are exemplified by cullin-Rbx E3s and APC/C. They consist of a RING-finger-containing subunit (RBX1 or RBX2) that functions to bind E2s, a scaffold-like cullin molecule, adaptor proteins, and a target recognizing subunit that binds substrates.

Class

Genetic Information Processing; Folding, sorting and degradation

Pathway map

ko04120

Ubiquitin mediated proteolysis

Module

M00379	SCF-MET30 complex [PATH:ko04120]
M00380	SCF-BTRC complex [PATH:ko04120]
M00381	SCF-SKP2 complex [PATH:ko04120]
M00382	SCF-FBS complex [PATH:ko04120]
M00383	ECV complex [PATH:ko04120]
M00384	Cul3-SPOP complex [PATH:ko04120]
M00385	Cul4-DDB1-DDB2 complex [PATH:ko04120]
M00386	Cul4-DDB1-CSA complex [PATH:ko04120]
M00387	SCF-FBW7 complex [PATH:ko04120]
M00388	ECS complex [PATH:ko04120]
M00389	APC/C complex [PATH:ko04120]
M00407	SCF-CDC4 complex [PATH:ko04120]
M00411	SCF-GRR1 complex [PATH:ko04120]

Disease

H00478	Prader-Willi and Angelman syndromes
H00509	3M syndrome
H00559	von Hippel-Lindau syndrome
H00583	Opitz-GBBB syndrome
H00593	Limb-girdle muscular dystrophy (LGMD)
H00658	Syndromic X-linked mental retardation
H00810	Progressive myoclonic epilepsy (PME)
H01232	Syndromic multisystem autoimmune disease
H01289	Mulibrey nanism

Other DBs

BSID:

466

GO:

0016567

Orthology

K03178	ubiquitin-activating enzyme E1 [EC:6.3.2.19]
K10684	ubiquitin-like 1-activating enzyme E1 A [EC:6.3.2.19]
K10685	ubiquitin-like 1-activating enzyme E1 B [EC:6.3.2.19]
K10686	ubiquitin-activating enzyme E1 C [EC:6.3.2.19]
K10698	ubiquitin-activating enzyme E1-like [EC:6.3.2.19]
K10699	ubiquitin-activating enzyme E1-like protein 2 [EC:6.3.2.19]
K10573	ubiquitin-conjugating enzyme E2 A [EC:6.3.2.19]
K10574	ubiquitin-conjugating enzyme E2 B [EC:6.3.2.19]
K06688	ubiquitin-conjugating enzyme E2 C [EC:6.3.2.19]
K06689	ubiquitin-conjugating enzyme E2 D/E [EC:6.3.2.19]
K10687	ubiquitin-conjugating enzyme E2 F [EC:6.3.2.19]
K10575	ubiquitin-conjugating enzyme E2 G1 [EC:6.3.2.19]
K04555	ubiquitin-conjugating enzyme E2 G2 [EC:6.3.2.19]
K10576	ubiquitin-conjugating enzyme E2 H [EC:6.3.2.19]
K10577	ubiquitin-conjugating enzyme E2 I [EC:6.3.2.19]
K10578	ubiquitin-conjugating enzyme E2 J1 [EC:6.3.2.19]
K04554	ubiquitin-conjugating enzyme E2 J2 [EC:6.3.2.19]
K04552	ubiquitin-conjugating enzyme E2 L3 [EC:6.3.2.19]
K04553	ubiquitin-conjugating enzyme E2 L6 [EC:6.3.2.19]
K10579	ubiquitin-conjugating enzyme E2 M [EC:6.3.2.19]
K10580	ubiquitin-conjugating enzyme E2 N [EC:6.3.2.19]
K10581	ubiquitin-conjugating enzyme E2 O [EC:6.3.2.19]
K10582	ubiquitin-conjugating enzyme E2 Q [EC:6.3.2.19]
K02207	ubiquitin-conjugating enzyme E2 R [EC:6.3.2.19]
K10583	ubiquitin-conjugating enzyme E2 S [EC:6.3.2.19]
K10584	ubiquitin-conjugating enzyme E2 U [EC:6.3.2.19]
K10688	ubiquitin-conjugating enzyme E2 W [EC:6.3.2.19]
K10585	ubiquitin-conjugating enzyme E2 Z [EC:6.3.2.19]
K04649	ubiquitin-conjugating enzyme (huntingtin interacting protein 2) [EC:6.3.2.19]
K10586	baculoviral IAP repeat-containing protein 6 (apollon) [EC:6.3.2.19]
K10587	ubiquitin-protein ligase E3 A [EC:6.3.2.19]
K10588	ubiquitin-protein ligase E3 B [EC:6.3.2.19]
K10589	ubiquitin-protein ligase E3 C [EC:6.3.2.19]
K04678	E3 ubiquitin ligase SMURF1/2 [EC:6.3.2.19]
K05632	atrophin-1 interacting protein 4 [EC:6.3.2.19]
K05633	atrophin-1 interacting protein 5 (WW domain containing E3 ubiquitin protein ligase 1) [EC:6.3.2.19]
K05630	atrophin-1 interacting protein 2 (WW domain containing E3 ubiquitin protein ligase 2) [EC:6.3.2.19]
K10590	E3 ubiquitin-protein ligase TRIP12 [EC:6.3.2.19]
K10591	E3 ubiquitin-protein ligase NEDD4 [EC:6.3.2.19]
K13305	E3 ubiquitin-protein ligase NEDD4-like [EC:6.3.2.19]
K10592	E3 ubiquitin-protein ligase HUWE1 [EC:6.3.2.19]
K10593	E3 ubiquitin-protein ligase EDD1 [EC:6.3.2.19]
K10594	E3 ubiquitin-protein ligase HERC1 [EC:6.3.2.19]
K10595	E3 ubiquitin-protein ligase HERC2 [EC:6.3.2.19]
K10614	E3 ubiquitin-protein ligase HERC3 [EC:6.3.2.19]
K10615	E3 ubiquitin-protein ligase HERC4 [EC:6.3.2.19]
K10596	ubiquitin conjugation factor E4 A [EC:6.3.2.19]
K10597	ubiquitin conjugation factor E4 B [EC:6.3.2.19]
K09561	STIP1 homology and U-box containing protein 1 [EC:6.3.2.19]
K10598	peptidyl-prolyl cis-trans isomerase-like 2 [EC:5.2.1.8]
K10599	pre-mRNA-processing factor 19 [EC:6.3.2.19]
K10600	U-box domain-containing protein 5
K06643	E3 ubiquitin-protein ligase Mdm2 [EC:6.3.2.19]
K04707	E3 ubiquitin-protein ligase CBL [EC:6.3.2.19]
K04556	parkin [EC:6.3.2.19]
K04506	E3 ubiquitin-protein ligase SIAH1 [EC:6.3.2.19]
K10054	probable transcription factor PML
K03175	TNF receptor-associated factor 6
K04416	mitogen-activated protein kinase kinase kinase 1 [EC:2.7.11.25]
K10143	E3 ubiquitin-protein ligase RFWD2 [EC:6.3.2.19]
K10144	RING finger and CHY zinc finger domain-containing protein 1 [EC:6.3.2.19]
K16060	baculoviral IAP repeat-containing protein 2/3
K04725	E3 ubiquitin-protein ligase XIAP
K16061	baculoviral IAP repeat-containing protein 7/8
K04706	E3 SUMO-protein ligase PIAS1 [EC:6.3.2.-]
K16063	E3 SUMO-protein ligase PIAS2 [EC:6.3.2.-]
K16064	E3 SUMO-protein ligase PIAS3 [EC:6.3.2.-]
K16065	E3 SUMO-protein ligase PIAS4 [EC:6.3.2.-]
K10601	E3 ubiquitin-protein ligase synoviolin [EC:6.3.2.19]
K10602	E3 ubiquitin-protein ligase NHLRC1 [EC:6.3.2.19]
K10603	autoimmune regulator
K10604	E3 ubiquitin-protein ligase MGRN1 [EC:6.3.2.19]
K10605	breast cancer type 1 susceptibility protein
K10606	E3 ubiquitin-protein ligase FANCL [EC:6.3.2.19]
K08285	midline 1 [EC:6.3.2.19]
K10607	tripartite motif-containing protein 32 [EC:6.3.2.19]
K10608	tripartite motif-containing protein 37
K03868	RING-box protein 1
K03347	cullin 1
K03094	S-phase kinase-associated protein 1
K03362	F-box and WD-40 domain protein 1/11
K03875	F-box and leucine-rich repeat protein 1 (S-phase kinase-associated protein 2)
K10260	F-box and WD-40 domain protein 7
K10099	F-box protein 2
K10291	F-box protein 4
K03361	F-box and WD-40 domain protein CDC4
K10259	F-box and WD-40 domain protein MET30
K03360	F-box and leucine-rich repeat protein GRR1
K03870	cullin 2
K03872	transcription elongation factor B, polypeptide 1
K03873	transcription elongation factor B, polypeptide 2
K03871	von Hippel-Lindau disease tumor supressor
K03869	cullin 3
K10456	kelch-like protein 19
K10447	kelch-like protein 9/13
K07868	Rho-related BTB domain-containing protein 1/2
K03876	BTB domain ubiquitin protein ligase cofactor
K10609	cullin 4
K10610	DNA damage-binding protein 1
K10140	DNA damage-binding protein 2
K10570	DNA excision repair protein ERCC-8
K10571	de-etiolated-1
K10611	RING-box protein 2
K10612	cullin 5
K04694	suppressor of cytokine signaling 1
K04696	suppressor of cytokine signaling 3
K10613	cullin 7
K10264	F-box and WD-40 domain protein 8
K03358	anaphase-promoting complex subunit 11
K03349	anaphase-promoting complex subunit 2
K03363	cell division cycle 20, cofactor of APC complex
K03364	cell division cycle 20-like protein 1, cofactor of APC complex
K03348	anaphase-promoting complex subunit 1
K03350	anaphase-promoting complex subunit 3
K03351	anaphase-promoting complex subunit 4
K03352	anaphase-promoting complex subunit 5
K03353	anaphase-promoting complex subunit 6
K03354	anaphase-promoting complex subunit 7
K03355	anaphase-promoting complex subunit 8
K03356	anaphase-promoting complex subunit 9
K03357	anaphase-promoting complex subunit 10
K03359	anaphase-promoting complex subunit 12
K12456	anaphase-promoting complex subunit 13
K12416	anaphase-promoting complex subunit SWM1

Reference

PMID:16922370 (Japanese)

Authors

Kamura T.

Title

[Cullin-based E3 family] Japanese

Journal

Tanpakushitsu Kakusan Koso 51:1167-72 (2006)

Reference

PMID:16922408 (Japanese)

Authors

Matsuda N.

Title

[Overview: hereditary disorders caused by dysfunction of ubiquitylation]

Journal

Tanpakushitsu Kakusan Koso 51:1402-7 (2006)

Reference

PMID:16922367 (Japanese)

Authors

Tokunaga F.

Title

[E2 ubiquitin-conjugating enzymes: structures and functions]

Journal

Tanpakushitsu Kakusan Koso 51:1150-6 (2006)

Reference

PMID:16816840

Authors

Nalepa G, Rolfe M, Harper JW.

Title

Drug discovery in the ubiquitin-proteasome system.

Journal

Nat Rev Drug Discov 5:596-613 (2006)

Reference

PMID:16405856

Authors

Hsu YJ, Goodman SR.

Title

Spectrin and ubiquitination: a review.

Journal

Cell Mol Biol (Noisy-le-grand) Suppl 51:OL801-7 (2005)

Reference

PMID:12944364

Authors

Hatakeyama S, Nakayama KI.

Title

Ubiquitylation as a quality control system for intracellular proteins.

Journal

J Biochem 134:1-8 (2003)

Reference

PMID:17588513

Authors

Lee J, Zhou P.

Title

DCAFs, the missing link of the CUL4-DDB1 ubiquitin ligase.

Journal

Mol Cell 26:775-80 (2007)

All links

Ontology (2)   
   KEGG BRITE (1)   
   GO (1)   
Pathway (16)   
   KEGG PATHWAY (2)   
   KEGG MODULE (13)   
   BioSystems (1)   
Disease (9)   
   KEGG DISEASE (9)   
Chemical reaction (4)   
   KEGG ENZYME (4)   
Gene (124)   
   KEGG ORTHOLOGY (124)   
Literature (7)   
   PubMed (7)   
All databases (162)   

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