KEGG   PATHWAY: ko04120Help
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
BRITE hierarchy
Pathway map
Ubiquitin mediated proteolysis
ko04120

All organismsOrtholog table
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: 
GO: 
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 [EC:6.3.2.19]
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
  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
  Authors
Hsu YJ, Goodman SR.
  Title
Spectrin and ubiquitination: a review.
  Journal
Cell Mol Biol (Noisy-le-grand) Suppl 51:OL801-7 (2005)
Reference
  Authors
Hatakeyama S, Nakayama KI.
  Title
Ubiquitylation as a quality control system for intracellular proteins.
  Journal
J Biochem 134:1-8 (2003)
Reference
  Authors
Lee J, Zhou P.
  Title
DCAFs, the missing link of the CUL4-DDB1 ubiquitin ligase.
  Journal
Mol Cell 26:775-80 (2007)

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