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
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
Database Links
Specialist databases
MEROPS T01.015 (Hs)
Other databases
BRENDA
CATH/Gene3D
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
GenitoUrinary Development Molecular Anatomy Project
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
Immuno Process ID:  8
Comment: 
GO Annotation:  Associated to GO processes
GO Processes:  regulation of hematopoietic stem cell differentiation (GO:1902036) TAS
References: 
Immuno Process:  Inflammation
Immuno Process ID:  2
Comment: 
GO Annotation:  Associated to GO processes
GO Processes:  stimulatory C-type lectin receptor signaling pathway (GO:0002223) TAS
type I interferon signaling pathway (GO:0060337) TAS
References: 
Immuno Process:  Antigen presentation
Immuno Process ID:  3
Comment: 
GO Annotation:  Associated to GO processes
GO Processes:  antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependent (GO:0002479) TAS
References: 
Immuno Process:  Immune regulation
Immuno Process ID:  6
Comment: 
GO Annotation:  Associated to GO processes
GO Processes:  stimulatory C-type lectin receptor signaling pathway (GO:0002223) TAS
Fc-epsilon receptor signaling pathway (GO:0038095) TAS
T cell receptor signaling pathway (GO:0050852) TAS
regulation of hematopoietic stem cell differentiation (GO:1902036) TAS
References: 
Immuno Process:  Cytokine production & signalling
Immuno Process ID:  9
Comment: 
GO Annotation:  Associated to GO processes
GO Processes:  tumor necrosis factor-mediated signaling pathway (GO:0033209) TAS
type I interferon signaling pathway (GO:0060337) TAS
References: 
Immuno Process:  Cellular signalling
Immuno Process ID:  11
Comment: 
GO Annotation:  Associated to GO processes
GO Processes:  protein polyubiquitination (GO:0000209) TAS
stimulatory C-type lectin receptor signaling pathway (GO:0002223) TAS
anaphase-promoting complex-dependent catabolic process (GO:0031145) TAS
SCF-dependent proteasomal ubiquitin-dependent protein catabolic process (GO:0031146) TAS
Fc-epsilon receptor signaling pathway (GO:0038095) TAS
proteasome-mediated ubiquitin-dependent protein catabolic process (GO:0043161) TAS
T cell receptor signaling pathway (GO:0050852) TAS
negative regulation of ubiquitin-protein ligase activity involved in mitotic cell cycle (GO:0051436) TAS
positive regulation of ubiquitin-protein ligase activity involved in regulation of mitotic cell cycle transition (GO:0051437) TAS
References: 
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 Letters5 (4): 405-410.

5. Ferrington DA, Gregerson DS. (2012) Immunoproteasomes: structure, function, and antigen presentation. Prog Mol Biol Transl Sci109: 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 Rep5: 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 Dermatopathol37 (7): 517-22. [PMID:26091509]

How to cite this page

T1: Proteasome: proteasome subunit beta 8. Last modified on 16/02/2017. Accessed on 15/12/2017. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=2408.