IL11RA

Chr 9AR

interleukin 11 receptor subunit alpha

Also known as: CRSDA

The IL11RA protein functions as a receptor for interleukin-11 and is essential for normal development of craniofacial bones and teeth, where it restricts suture fusion and controls tooth number. Biallelic mutations cause autosomal recessive craniosynostosis and dental anomalies. The gene shows very low constraint against loss-of-function variants (pLI near zero), which is consistent with its recessive inheritance pattern.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.541 OMIM phenotype
Clinical SummaryIL11RA
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Gene-Disease Validity (ClinGen)
craniosynostosis and dental anomalies · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.54LOEUF
pLI 0.000
Z-score -0.61
OE 1.13 (0.841.54)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.64Z-score
OE missense 0.88 (0.790.99)
209 obs / 236.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.13 (0.841.54)
00.351.4
Missense OE0.88 (0.790.99)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 29 / 25.7Missense obs/exp: 209 / 236.6Syn Z: 0.12
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveIL11RA-related craniosynostosisLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6452th %ile
GOF
0.7125th %ile
LOF
0.3940th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

IL11RA · 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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