The protein contains two RNA recognition motifs that bind to RNA molecules as part of RNA processing pathways. Mutations cause autosomal recessive developmental and epileptic encephalopathy with onset in infancy, characterized by severe intellectual disability, seizures, and developmental regression. This gene shows tolerance to loss-of-function variation in the general population.

OMIMResearchSummary from RefSeq
DNmechanismLOEUF 1.43
Clinical SummaryRBM34
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
1.43LOEUF
pLI 0.000
Z-score -0.01
OE 1.00 (0.711.43)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.39Z-score
OE missense 1.07 (0.971.19)
251 obs / 234.1 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.00 (0.711.43)
00.351.4
Missense OE1.07 (0.971.19)
00.61.4
Synonymous OE1.19
01.21.6
LoF obs/exp: 22 / 21.9Missense obs/exp: 251 / 234.1Syn Z: -1.36
DN
0.7230th %ile
GOF
0.5759th %ile
LOF
0.4332th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

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.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RBM34 · 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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