HomeAboutRankingsData Sources
© 2026 GeneE
🧬
GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
ⓘGeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
PSMB11
proteasome subunit beta 11
Chromosome 14 · 14q11.2
NCBI Gene: 122706Ensembl: ENSG00000222028.5HGNC: HGNC:31963UniProt: A5LHX3
19PubMed Papers
20Diseases
6Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Protease
CLINICAL
FDA Approved Target
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
proteolysisprotein bindingpeptidase activitynucleusmultiple myelomaMantle cell lymphomaneoplasmamyloidosis
✦AI Summary

PSMB11 encodes β5t, a thymus-specific catalytic subunit of the thymoproteasome that is essential for CD8+ T cell development. The thymoproteasome is a specialized 20S proteasome expressed exclusively in cortical thymic epithelial cells (cTECs) that generates a distinct set of MHC class I-bound peptides required for positive selection of functionally competent CD8+ T cells 1. PSMB11 arose through tandem duplication from PSMB5 in early jawed vertebrates and remains intronless, representing a unique evolutionary adaptation 2. Mechanistically, β5t reduces the chymotrypsin-like activity of the standard proteasome compared to its homologs PSMB5 and PSMB8, creating a specialized peptide repertoire that shapes T cell selection 3. PSMB11 expression is tightly regulated as a FOXN1 target gene in cTECs, and FOXN1 haploinsufficiency reduces PSMB11 expression and CD8+ T cell production 4. Genetic variations in PSMB11 significantly impact human health. Common human polymorphisms alter β5t amino acid sequences, reducing protein processing and CD8+ T cell cellularity in homozygotes, though severe health problems are rare 1. Computationally, PSMB11 is highly enriched for functional variants; specific polymorphisms impair CD8+ T cell development in vivo and associate with increased autoimmune disease risk 5, suggesting proteasome variations complement MHC haplotypes in determining individual susceptibility to autoimmunity.

Sources cited
1
PSMB11 encodes β5t subunit of thymoproteasome; human variants affect β5t processing and CD8+ T cell production
PMID: 28515360
2
PSMB11 gene arose by tandem duplication from PSMB5 in jawed vertebrates; is intronless and unique to thymoproteasomes
PMID: 21748441
3
β5t reduces chymotrypsin-like activity of proteasome; cTECs express thymoproteasome for T cell selection
PMID: 23612989
4
PSMB11 is a FOXN1 target gene; FOXN1 haploinsufficiency reduces PSMB11 expression and CD8+ T cell counts
PMID: 31447097
5
PSMB11 polymorphisms impair CD8+ T cell development and associate with autoimmune disease risk in humans
PMID: 28783658
6
PSMB11 mutations impact positive selection of CD8+ T cells in thymus development
PMID: 33537838
Disease Associationsⓘ20
multiple myelomaOpen Targets
0.60Moderate
Mantle cell lymphomaOpen Targets
0.50Moderate
neoplasmOpen Targets
0.50Moderate
amyloidosisOpen Targets
0.37Weak
glioblastoma multiformeOpen Targets
0.26Weak
brain glioblastomaOpen Targets
0.26Weak
lymphomaOpen Targets
0.11Weak
Waldenstrom macroglobulinemiaOpen Targets
0.11Weak
non-Hodgkins lymphomaOpen Targets
0.11Weak
non-small cell lung carcinomaOpen Targets
0.10Weak
AL amyloidosisOpen Targets
0.10Suggestive
leukemiaOpen Targets
0.10Suggestive
acute myeloid leukemiaOpen Targets
0.09Suggestive
chronic graft versus host diseaseOpen Targets
0.09Suggestive
myelodysplastic syndromeOpen Targets
0.08Suggestive
cancerOpen Targets
0.08Suggestive
small cell lung carcinomaOpen Targets
0.08Suggestive
autoimmune thrombocytopenic purpuraOpen Targets
0.07Suggestive
kidney cancerOpen Targets
0.07Suggestive
MALT lymphomaOpen Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Drug Targets6
BORTEZOMIB D-MANNITOLApproved
26S proteasome inhibitor
Mantle cell lymphoma
CARFILZOMIBApproved
26S proteasome inhibitor
multiple myeloma
IXAZOMIBApproved
26S proteasome inhibitor
multiple myeloma
IXAZOMIB CITRATEApproved
26S proteasome inhibitor
multiple myeloma
MARIZOMIBPhase III
20S proteasome inhibitor
brain glioblastoma
OPROZOMIBPhase II
26S proteasome inhibitor
Hodgkins lymphoma
Related Genes
PSMB3Protein interaction100%PSMB8Protein interaction100%PSMD14Protein interaction100%PSMB2Protein interaction100%PSMD7Protein interaction100%PSMB1Protein interaction100%
Tissue Expression6 tissues
Ovary
0%
Brain
0%
Heart
0%
Lung
0%
Liver
0%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
PSMB11PSMB3PSMB8PSMD14PSMB2PSMD7PSMB1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt A5LHX3
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.97LoF Tolerant
pLIⓘ
0.01Tolerant
Observed/Expected LoF4.69 [0.82–1.97]
RankingsWhere PSMB11 stands among ~20K protein-coding genes
  • #14,508of 20,598
    Most Researched19
  • #432of 1,025
    FDA-Approved Drug Targets4
  • #17,771of 17,882
    Most Constrained (LOEUF)1.97
Genes detectedPSMB11
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Thymus and autoimmunity.
PMID: 33537838
Semin Immunopathol · 2021
1.00
2
A human PSMB11 variant affects thymoproteasome processing and CD8+ T cell production.
PMID: 28515360
JCI Insight · 2017
0.90
3
Human thymoproteasome variations influence CD8 T cell selection.
PMID: 28783658
Sci Immunol · 2017
0.80
4
Comparative genomic analysis of the proteasome β5t subunit gene: implications for the origin and evolution of thymoproteasomes.
PMID: 21748441
Immunogenetics · 2012
0.70
5
Heterozygous FOXN1 Variants Cause Low TRECs and Severe T Cell Lymphopenia, Revealing a Crucial Role of FOXN1 in Supporting Early Thymopoiesis.
PMID: 31447097
Am J Hum Genet · 2019
0.60