IL2RB

Chr 22AR

interleukin 2 receptor subunit beta

Also known as: CD122, IL15RB, IMD63, P70-75

This gene encodes the beta subunit of the interleukin-2 receptor, which transduces mitogenic signals from IL-2 and mediates receptor-mediated endocytosis in T cell-mediated immune responses. Biallelic mutations cause autosomal recessive immunodeficiency 63 with lymphoproliferation and autoimmunity. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.496), reflecting its important role in immune system function.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismARLOEUF 0.501 OMIM phenotype
Clinical SummaryIL2RB
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Gene-Disease Validity (ClinGen)
immunodeficiency 63 with lymphoproliferation and autoimmunity · ARModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.24) despite low pLI — interpret in context.
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ClinVar Variants
34 unique Pathogenic / Likely Pathogenic· 217 VUS of 521 total submissions
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.50LOEUF
pLI 0.291
Z-score 3.26
OE 0.24 (0.120.50)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.44Z-score
OE missense 0.93 (0.841.02)
284 obs / 305.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.24 (0.120.50)
00.351.4
Missense OE0.93 (0.841.02)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 5 / 21.2Missense obs/exp: 284 / 305.7Syn Z: 0.18
DN
0.7035th %ile
GOF
0.78top 25%
LOF
0.3357th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function, dominant-negative and loss-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
DNprediction above median
LOF35% of P/LP variants are LoF · LOEUF 0.50

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

521 submitted variants in ClinVar

Classification Summary

Pathogenic26
Likely Pathogenic8
VUS217
Likely Benign229
Benign22
Conflicting8
26
Pathogenic
8
Likely Pathogenic
217
VUS
229
Likely Benign
22
Benign
8
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
5
2
19
0
26
Likely Pathogenic
7
0
1
0
8
VUS
7
197
10
3
217
Likely Benign
0
9
84
136
229
Benign
0
5
14
3
22
Conflicting
8
Total19213128142510

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

IL2RB · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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