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The proteasome is a multicatalytic proteinase complex with a highly ordered ring-shaped 20S core structure. The core structure is composed of 4 rings of 28 non-identical subunits; 2 rings are composed of 7 alpha subunits and 2 rings are composed of 7 beta subunits. Proteasomes are distributed throughout eukaryotic cells at a high concentration and cleave peptides in an ATP/ubiquitin-dependent process in a non-lysosomal pathway. An essential function of a modified proteasome, the immunoproteasome, is the processing of class I MHC peptides. This gene encodes a member of the proteasome B-type family, also known as the T1B family, that is a 20S core beta subunit. This gene is located in the class II region of the MHC (major histocompatibility complex). Expression of this gene is induced by gamma interferon and this gene product replaces catalytic subunit 1 (proteasome beta 6 subunit) in the immunoproteasome. Proteolytic processing is required to generate a mature subunit. [provided by RefSeq, Mar 2010]
PSMB9 (Proteasome 20S Subunit Beta 9) is a Protein Coding gene. Diseases associated with PSMB9 include Proteasome-Associated Autoinflammatory Syndrome 3 and Nasopharyngeal Disease. Among its related pathways are Regulation of activated PAK-2p34 by proteasome mediated degradation and Antigen processing-Cross presentation. Gene Ontology (GO) annotations related to this gene include threonine-type endopeptidase activity. An important paralog of this gene is PSMB6.
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0004175 | endopeptidase activity | IBA | 21873635 |
GO:0004298 | threonine-type endopeptidase activity | IEA | -- |
GO:0005515 | protein binding | IPI | 14733938 |
GO:0008233 | peptidase activity | IEA | -- |
GO:0016787 | hydrolase activity | IEA | -- |
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0000502 | proteasome complex | TAS | 8666937 |
GO:0005634 | nucleus | IBA | 21873635 |
GO:0005654 | nucleoplasm | TAS | -- |
GO:0005737 | cytoplasm | IBA | 21873635 |
GO:0005829 | cytosol | TAS | -- |
SuperPathway | Contained pathways | ||
---|---|---|---|
1 | Regulation of activated PAK-2p34 by proteasome mediated degradation |
.93
.93
|
.80
.79
.78
.69
.47
|
2 | RET signaling |
.92
|
.92
|
3 | Chks in Checkpoint Regulation |
Chks in Checkpoint Regulation
.58
G2-M Phase Transition
.58
Estrogen-mediated S-Phase Entry
.54
|
SREBP Proteolysis
.42
DNA Repair Mechanisms
.31
|
4 | HIV Life Cycle |
.65
|
.45
|
5 | Class I MHC mediated antigen processing and presentation |
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0000165 | MAPK cascade | TAS | -- |
GO:0000209 | protein polyubiquitination | TAS | -- |
GO:0002223 | stimulatory C-type lectin receptor signaling pathway | TAS | -- |
GO:0002376 | immune system process | IEA | -- |
GO:0002479 | antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependent | TAS | -- |
Name | Status | Disease Links | Group | Role | Mechanism of Action | Clinical Trials |
---|---|---|---|---|---|---|
carfilzomib | Approved, Investigational | Pharma | Target, inhibitor, other | Other, 20S Proteasome Inhibitor | 0 | |
Bortezomib | Approved, Investigational | Pharma | inhibitor, other | Proteosome and NF-kappaB inhibitor, Other, Proteasome inhibitors | 910 | |
Celastrol | Pharma | Antioxidant, anti-inflammatory and immunosuppressive agent | 0 | |||
Dihydroeponemycin | Pharma | Proteasome inhibitor,antitumor reagent,eponemycin ddrivative | 0 | |||
MLN2238 | Pharma | β5 site of the 20S proteasome inhibitor | 0 |
Compound | Action | Cas Number |
---|---|---|
Celastrol | Antioxidant, anti-inflammatory and immunosuppressive agent | 34157-83-0 |
Dihydroeponemycin | Proteasome inhibitor,antitumor reagent,eponemycin ddrivative | 126463-64-7 |
MLN2238 | β5 site of the 20S proteasome inhibitor | 1072833-77-2 |
ONX-0914 (PR-957) | Immunoproteasome inhibitor,potent and selective | 960374-59-8 |
This gene was present in the common ancestor of animals and fungi.
Organism | Taxonomy | Gene | Similarity | Type | Details |
---|---|---|---|---|---|
Chimpanzee (Pan troglodytes) |
Mammalia | PSMB9 30 31 |
|
OneToOne | |
Dog (Canis familiaris) |
Mammalia | PSMB9 30 31 |
|
OneToOne | |
Cow (Bos Taurus) |
Mammalia | PSMB9 30 31 |
|
OneToOne | |
Rat (Rattus norvegicus) |
Mammalia | Psmb9 30 |
|
||
Mouse (Mus musculus) |
Mammalia | Psmb9 30 17 31 |
|
OneToOne | |
Oppossum (Monodelphis domestica) |
Mammalia | PSMB9 31 |
|
OneToOne | |
Lizard (Anolis carolinensis) |
Reptilia | PSMB9 31 |
|
OneToOne | |
Tropical Clawed Frog (Silurana tropicalis) |
Amphibia | psmb9 30 |
|
||
Zebrafish (Danio rerio) |
Actinopterygii | psmb9a 30 31 |
|
OneToOne | |
-- 30 |
|
||||
Rainbow Trout (Oncorhynchus mykiss) |
Actinopterygii | Omy.11923 30 |
|
||
Fruit Fly (Drosophila melanogaster) |
Insecta | Prosbeta1 31 |
|
OneToMany | |
Worm (Caenorhabditis elegans) |
Secernentea | pbs-1 31 |
|
OneToMany | |
Baker's yeast (Saccharomyces cerevisiae) |
Saccharomycetes | PRE3 31 |
|
OneToMany | |
Sea Squirt (Ciona savignyi) |
Ascidiacea | CSA.5051 31 |
|
OneToMany |
SNP ID | Clinical significance and condition | Chr 06 pos | Variation | AA Info | Type |
---|---|---|---|---|---|
756788 | Likely Benign: not provided | 32,856,170(+) | C/T | SYNONYMOUS_VARIANT | |
rs367733308 | Uncertain Significance: not provided | 32,858,075(+) | C/A | SYNONYMOUS_VARIANT | |
rs369359789 | Pathogenic: PROTEASOME-ASSOCIATED AUTOINFLAMMATORY SYNDROME 3. Proteasome-associated autoinflammatory syndrome 3 (PRAAS3) [MIM:617591] | 32,858,467(+) | G/Ap.Gly165Asp | MISSENSE_VARIANT | |
rs17213861 | - | p.Arg173Cys | |||
rs17587 | - | p.Arg60His |
Variant ID | Type | Subtype | PubMed ID |
---|---|---|---|
nsv1073969 | CNV | deletion | 25765185 |
nsv1112900 | CNV | deletion | 24896259 |
nsv1119076 | CNV | deletion | 24896259 |
nsv830633 | CNV | loss | 17160897 |
Disorder | Aliases | PubMed IDs |
---|---|---|
proteasome-associated autoinflammatory syndrome 3 |
|