PSMB2

Chr 1

proteasome 20S subunit beta 2

The PSMB2 protein is a non-catalytic beta subunit of the 20S core proteasome complex that degrades intracellular proteins to maintain cellular protein homeostasis and generate MHC class I antigenic peptides. Mutations cause autosomal recessive proteasome-associated autoinflammatory syndrome, characterized by early-onset immunodeficiency, autoinflammation, and dermatitis. This gene is highly constrained against loss-of-function variants, indicating its essential cellular role.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.39
Clinical SummaryPSMB2
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.91). One damaged copy is likely sufficient to cause disease.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.39LOEUF
pLI 0.914
Z-score 2.98
OE 0.08 (0.030.39)
Highly constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.26Z-score
OE missense 0.68 (0.570.81)
82 obs / 120.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.08 (0.030.39)
00.351.4
Missense OE0.68 (0.570.81)
00.61.4
Synonymous OE0.69
01.21.6
LoF obs/exp: 1 / 12.3Missense obs/exp: 82 / 120.9Syn Z: 1.62

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PSMB2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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