KEGG   PATHWAY: hsa03050Help
Entry
hsa03050                    Pathway                                

Name
Proteasome - Homo sapiens (human)
Description
The proteasome is a protein-destroying apparatus involved in many essential cellular functions, such as regulation of cell cycle, cell differentiation, signal transduction pathways, antigen processing for appropriate immune responses, stress signaling, inflammatory responses, and apoptosis. It is capable of degrading a variety of cellular proteins in a rapid and timely fashion and most substrate proteins are modified by ubiquitin before their degradation by the proteasome. The proteasome is a large protein complex consisting of a proteolytic core called the 20S particle and ancillary factors that regulate its activity in various ways. The most common form is the 26S proteasome containing one 20S core particle and two 19S regulatory particles that enable the proteasome to degrade ubiquitinated proteins by an ATP-dependent mechanism. Another form is the immunoproteasome containing two 11S regulatory particles, PA28 alpha and PA28 beta, which are induced by interferon gamma under the conditions of intensified immune response. Other regulatory particles include PA28 gamma and PA200. Although PA28 gamma also belongs to a family of activators of the 20S proteasome, it is localized within the nucleus and forms a homoheptamer. PA28 gamma has been implicated in the regulation of cell cycle progression and apoptosis. PA200 has been identified as a large nuclear protein that stimulates proteasomal hydrolysis of peptides.
Class
Genetic Information Processing; Folding, sorting and degradation
BRITE hierarchy
Pathway map
Proteasome
hsa03050

All organismsOrtholog table
Module
Immunoproteasome [PATH:hsa03050]
Proteasome, 20S core particle [PATH:hsa03050]
Proteasome, 19S regulatory particle (PA700) [PATH:hsa03050]
Disease
H00790  
Keratosis linearis with ichthyosis congenita and sclerosing keratoderma
Drug
D03150  
Bortezomib (JAN/USAN/INN)
D08880  
Carfilzomib (JAN/USAN/INN)
D09640  
Marizomib (USAN/INN)
D10110  
Delanzomib (USAN)
D10130  
Ixazomib (USAN)
D10131  
Ixazomib citrate (USAN)
Other DBs
BSID: 
GO: 
Organism
Homo sapiens (human) [GN:hsa]
Gene
5709  
PSMD3; proteasome (prosome, macropain) 26S subunit, non-ATPase, 3 [KO:K03033]
5718  
PSMD12; proteasome (prosome, macropain) 26S subunit, non-ATPase, 12 [KO:K03035]
5717  
PSMD11; proteasome (prosome, macropain) 26S subunit, non-ATPase, 11 [KO:K03036]
9861  
PSMD6; proteasome (prosome, macropain) 26S subunit, non-ATPase, 6 [KO:K03037]
5713  
PSMD7; proteasome (prosome, macropain) 26S subunit, non-ATPase, 7 [KO:K03038]
5719  
PSMD13; proteasome (prosome, macropain) 26S subunit, non-ATPase, 13 [KO:K03039]
10213  
PSMD14; proteasome (prosome, macropain) 26S subunit, non-ATPase, 14 [KO:K03030]
5714  
PSMD8; proteasome (prosome, macropain) 26S subunit, non-ATPase, 8 [KO:K03031]
7979  
SHFM1; split hand/foot malformation (ectrodactyly) type 1 [KO:K10881]
5710  
PSMD4; proteasome (prosome, macropain) 26S subunit, non-ATPase, 4 [KO:K03029]
5708  
PSMD2; proteasome (prosome, macropain) 26S subunit, non-ATPase, 2 [KO:K03028]
5707  
PSMD1; proteasome (prosome, macropain) 26S subunit, non-ATPase, 1 [KO:K03032]
5701  
PSMC2; proteasome (prosome, macropain) 26S subunit, ATPase, 2 [KO:K03061]
5700  
PSMC1; proteasome (prosome, macropain) 26S subunit, ATPase, 1 [KO:K03062]
5705  
PSMC5; proteasome (prosome, macropain) 26S subunit, ATPase, 5 [KO:K03066]
5706  
PSMC6; proteasome (prosome, macropain) 26S subunit, ATPase, 6 [KO:K03064]
5702  
PSMC3; proteasome (prosome, macropain) 26S subunit, ATPase, 3 [KO:K03065]
5704  
PSMC4; proteasome (prosome, macropain) 26S subunit, ATPase, 4 [KO:K03063]
5720  
PSME1; proteasome (prosome, macropain) activator subunit 1 (PA28 alpha) [KO:K06696]
5721  
PSME2; proteasome (prosome, macropain) activator subunit 2 (PA28 beta) [KO:K06697]
10197  
PSME3; proteasome (prosome, macropain) activator subunit 3 (PA28 gamma [KO:K06698]
23198  
PSME4; proteasome (prosome, macropain) activator subunit 4 [KO:K06699]
5687  
PSMA6; proteasome (prosome, macropain) subunit, alpha type, 6 [KO:K02730] [EC:3.4.25.1]
5683  
PSMA2; proteasome (prosome, macropain) subunit, alpha type, 2 [KO:K02726] [EC:3.4.25.1]
5685  
PSMA4; proteasome (prosome, macropain) subunit, alpha type, 4 [KO:K02728] [EC:3.4.25.1]
5688  
PSMA7; proteasome (prosome, macropain) subunit, alpha type, 7 [KO:K02731] [EC:3.4.25.1]
143471  
PSMA8; proteasome (prosome, macropain) subunit, alpha type, 8 [KO:K02731] [EC:3.4.25.1]
5686  
PSMA5; proteasome (prosome, macropain) subunit, alpha type, 5 [KO:K02729] [EC:3.4.25.1]
5682  
PSMA1; proteasome (prosome, macropain) subunit, alpha type, 1 [KO:K02725] [EC:3.4.25.1]
5684  
PSMA3; proteasome (prosome, macropain) subunit, alpha type, 3 [KO:K02727] [EC:3.4.25.1]
5694  
PSMB6; proteasome (prosome, macropain) subunit, beta type, 6 [KO:K02738] [EC:3.4.25.1]
5695  
PSMB7; proteasome (prosome, macropain) subunit, beta type, 7 [KO:K02739] [EC:3.4.25.1]
5691  
PSMB3; proteasome (prosome, macropain) subunit, beta type, 3 [KO:K02735] [EC:3.4.25.1]
5690  
PSMB2; proteasome (prosome, macropain) subunit, beta type, 2 [KO:K02734] [EC:3.4.25.1]
5693  
PSMB5; proteasome (prosome, macropain) subunit, beta type, 5 [KO:K02737] [EC:3.4.25.1]
5689  
PSMB1; proteasome (prosome, macropain) subunit, beta type, 1 [KO:K02732] [EC:3.4.25.1]
5692  
PSMB4; proteasome (prosome, macropain) subunit, beta type, 4 [KO:K02736] [EC:3.4.25.1]
5698  
PSMB9; proteasome (prosome, macropain) subunit, beta type, 9 [KO:K02741] [EC:3.4.25.1]
5699  
PSMB10; proteasome (prosome, macropain) subunit, beta type, 10 [KO:K02733] [EC:3.4.25.1]
5696  
PSMB8; proteasome (prosome, macropain) subunit, beta type, 8 [KO:K02740] [EC:3.4.25.1]
122706  
PSMB11; proteasome (prosome, macropain) subunit, beta type, 11 [KO:K11598] [EC:3.4.25.1]
3458  
IFNG; interferon, gamma [KO:K04687]
9491  
PSMF1; proteasome (prosome, macropain) inhibitor subunit 1 (PI31) [KO:K06700]
51371  
POMP; proteasome maturation protein [KO:K11599]
Reference
  Authors
Hirano Y, Murata S, Tanaka K
  Title
Large- and small-scale purification of mammalian 26S proteasomes.
  Journal
Methods Enzymol 399:227-40 (2005)
Reference
  Authors
Saeki Y, Tanaka K
  Title
Cell biology: two hands for degradation.
  Journal
Nature 453:460-1 (2008)
Reference
  Authors
Smith DM, Benaroudj N, Goldberg A
  Title
Proteasomes and their associated ATPases: a destructive combination.
  Journal
J Struct Biol 156:72-83 (2006)
Reference
  Authors
Strehl B, Seifert U, Kruger E, Heink S, Kuckelkorn U, Kloetzel PM
  Title
Interferon-gamma, the functional plasticity of the ubiquitin-proteasome system, and MHC class I antigen processing.
  Journal
Immunol Rev 207:19-30 (2005)
Reference
  Authors
Isono E, Toh-e A
  Title
[Biogenesis of the 26S proteasome in the yeast Saccharomyces cerevisiae]
  Journal
Tanpakushitsu Kakusan Koso 51:1224-9 (2006)
Reference
  Authors
Hirano Y, Murata S
  Title
[Mechanism of the assembly of mammalian 20S proteasome]
  Journal
Tanpakushitsu Kakusan Koso 51:1230-5 (2006)
Reference
  Authors
Saeki Y, Yashiroda H
  Title
[Diversity of proteasome activators]
  Journal
Tanpakushitsu Kakusan Koso 51:1236-40 (2006)
Reference
  Authors
Darwin KH
  Title
Prokaryotic ubiquitin-like protein (Pup), proteasomes and pathogenesis.
  Journal
Nat Rev Microbiol 7:485-91 (2009)
KO pathway
 

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