proteasome subunit beta 8 | T1: Proteasome | IUPHAR Guide to IMMUNOPHARMACOLOGY

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proteasome subunit beta 8

  Target has curated data in GtoImmuPdb

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

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
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
Human Protein Atlas
KEGG Enzyme
RefSeq Nucleotide
RefSeq Protein
Enzyme Reaction
EC Number:

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

Key to terms and symbols View all chemical structures Click column headers to sort
Ligand Sp. Action Affinity Parameter 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:  Inflammation
GO Annotations:  Associated to 1 GO processes
GO:0060337 type I interferon signaling pathway TAS
Immuno Process:  Antigen presentation
GO Annotations:  Associated to 1 GO processes
click arrow to show/hide IEA associations
GO:0019882 antigen processing and presentation IEA
Immuno Process:  Cytokine production & signalling
GO Annotations:  Associated to 1 GO processes
GO:0060337 type I interferon signaling pathway 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


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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 25/05/2019. IUPHAR/BPS Guide to PHARMACOLOGY,