proteasome subunit beta 8

Target id: 2408

Nomenclature: proteasome subunit beta 8

Family: T1: Proteasome

Annotation status:  image of a grey circle Awaiting annotation/under development. Please contact us if you can help with annotation.  » Email us

   GtoImmuPdb view: OFF :     proteasome subunit beta 8 has curated GtoImmuPdb data

Gene and Protein Information
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human - 276 6p21.3 PSMB8 proteasome subunit beta 8
Mouse - 276 17 B1 Psmb8 proteasome (prosome, macropain) subunit, beta type 8 (large multifunctional peptidase 7)
Rat - 276 20p12 Psmb8 proteasome subunit beta 8
Previous and Unofficial Names
large multifunctional protease 7 | Lmp7 | low molecular mass protein 7 | macropain subunit C13 | multicatalytic endopeptidase complex subunit C13 | proteasome component C13 | proteasome subunit beta-5i | proteasome subunit MC13 | Ring10 | proteasome (prosome, macropain) subunit, beta type, 8 | proteasome (prosome
Database Links
Specialist databases
MEROPS T01.015 (Hs)
Other databases
BRENDA
CATH/Gene3D
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
Human Protein Atlas
KEGG Enzyme
KEGG Gene
OMIM
Orphanet
RefSeq Nucleotide
RefSeq Protein
UniProtKB
Wikipedia
Enzyme Reaction
EC Number: 3.4.25.1

Download all structure-activity data for this target as a CSV file

Inhibitors
Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Affinity Units Reference
HT1042 Hs Inhibition 6.4 pKi 4
pKi 6.4 (Ki 4.2x10-7 M) [4]
ONX-0914 Hs Inhibition 7.0 pIC50 7
pIC50 7.0 (IC50 1x10-7 M) [7]
Immunopharmacology Comments
PSMB8 is a component of the immunoproteasome [5]. It is a IFN-γ-inducible gene [1], expressed by immune and non-immune cell types [6]. Genetic mutations in PSMB8 [2-3] have been identified in patients with the autoinflammatory disorder, CANDLE syndrome [8].
Immuno Process Associations
Immuno Process:  Immune system development
GO Annotations:  Associated to 1 GO processes
GO:1902036 regulation of hematopoietic stem cell differentiation TAS
Immuno Process:  Inflammation
GO Annotations:  Associated to 2 GO processes
GO:0002223 stimulatory C-type lectin receptor signaling pathway TAS
GO:0060337 type I interferon signaling pathway TAS
Immuno Process:  Antigen presentation
GO Annotations:  Associated to 1 GO processes
GO:0002479 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependent TAS
Immuno Process:  Immune regulation
GO Annotations:  Associated to 4 GO processes
GO:0002223 stimulatory C-type lectin receptor signaling pathway TAS
GO:0038095 Fc-epsilon receptor signaling pathway TAS
GO:0050852 T cell receptor signaling pathway TAS
GO:1902036 regulation of hematopoietic stem cell differentiation TAS
Immuno Process:  Cytokine production & signalling
GO Annotations:  Associated to 2 GO processes
GO:0033209 tumor necrosis factor-mediated signaling pathway TAS
GO:0060337 type I interferon signaling pathway TAS
Immuno Process:  Cellular signalling
GO Annotations:  Associated to 9 GO processes
GO:0000209 protein polyubiquitination TAS
GO:0002223 stimulatory C-type lectin receptor signaling pathway TAS
GO:0031145 anaphase-promoting complex-dependent catabolic process TAS
GO:0031146 SCF-dependent proteasomal ubiquitin-dependent protein catabolic process TAS
GO:0038095 Fc-epsilon receptor signaling pathway TAS
GO:0043161 proteasome-mediated ubiquitin-dependent protein catabolic process TAS
GO:0050852 T cell receptor signaling pathway TAS
GO:0051436 negative regulation of ubiquitin-protein ligase activity involved in mitotic cell cycle TAS
GO:0051437 positive regulation of ubiquitin-protein ligase activity involved in regulation of mitotic cell cycle transition TAS
Clinically-Relevant Mutations and Pathophysiology
Disease:  CANDLE syndrome
Synonyms: Autoinflammation, lipodystrophy, and dermatosis syndrome [OMIM: 256040]
Chronic atypical neutrophilic dermatosis-lipodystrophy-elevated temperature syndrome [Orphanet: ORPHA325004]
OMIM: 256040
Orphanet: ORPHA325004
Disease:  JMP syndrome
Synonyms: Autoinflammation, lipodystrophy, and dermatosis syndrome [OMIM: 256040]
Joint contractures-muscular atrophy-microcytic anemia-panniculitis-associated lipodystrophy syndrome [Orphanet: ORPHA324999]
OMIM: 256040
Orphanet: ORPHA324999
Disease:  Nakajo-Nishimura syndrome
Synonyms: Amyotrophy - fat tissue anomaly [Orphanet: ORPHA2615]
Autoinflammation, lipodystrophy, and dermatosis syndrome [OMIM: 256040]
Secondary hypertrophic osteoperiostosis with pernio [Orphanet: ORPHA2615]
OMIM: 256040
Orphanet: ORPHA2615

References

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1. Basler M, Kirk CJ, Groettrup M. (2013) The immunoproteasome in antigen processing and other immunological functions. Curr. Opin. Immunol., 25 (1): 74-80. [PMID:23219269]

2. Brehm A, Liu Y, Sheikh A, Marrero B, Omoyinmi E, Zhou Q, Montealegre G, Biancotto A, Reinhardt A, Almeida de Jesus A et al.. (2015) Additive loss-of-function proteasome subunit mutations in CANDLE/PRAAS patients promote type I IFN production. J. Clin. Invest., 125 (11): 4196-211. [PMID:26524591]

3. Cavalcante MP, Brunelli JB, Miranda CC, Novak GV, Malle L, Aikawa NE, Jesus AA, Silva CA. (2016) CANDLE syndrome: chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature-a rare case with a novel mutation. Eur. J. Pediatr., 175 (5): 735-40. [PMID:26567544]

4. Fan H, Angelo NG, Warren JD, Nathan CF, Lin G. (2014) Oxathiazolones Selectively Inhibit the Human Immunoproteasome over the Constitutive Proteasome. ACS Medicinal Chemistry Letters, 5 (4): 405-410.

5. Ferrington DA, Gregerson DS. (2012) Immunoproteasomes: structure, function, and antigen presentation. Prog Mol Biol Transl Sci, 109: 75-112. [PMID:22727420]

6. Keller IE, Vosyka O, Takenaka S, Kloß A, Dahlmann B, Willems LI, Verdoes M, Overkleeft HS, Marcos E, Adnot S et al.. (2015) Regulation of immunoproteasome function in the lung. Sci Rep, 5: 10230. [PMID:25989070]

7. Muchamuel T, Basler M, Aujay MA, Suzuki E, Kalim KW, Lauer C, Sylvain C, Ring ER, Shields J, Jiang J et al.. (2009) A selective inhibitor of the immunoproteasome subunit LMP7 blocks cytokine production and attenuates progression of experimental arthritis. Nat. Med., 15 (7): 781-7. [PMID:19525961]

8. Torrelo A, Colmenero I, Requena L, Paller AS, Ramot Y, Richard Lee CC, Vera A, Zlotogorski A, Goldbach-Mansky R, Kutzner H. (2015) Histologic and Immunohistochemical Features of the Skin Lesions in CANDLE Syndrome. Am J Dermatopathol, 37 (7): 517-22. [PMID:26091509]

How to cite this page

T1: Proteasome: proteasome subunit beta 8. Last modified on 16/02/2017. Accessed on 20/07/2018. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=2408.