LARP1B

Chr 4

La ribonucleoprotein 1B

Also known as: LARP2

This gene encodes an RNA-binding protein containing La motifs that fold into RNA recognition motifs. Mutations cause autosomal recessive intellectual disability with speech delay and dysmorphic features, typically presenting in early childhood. The gene is highly constrained against loss-of-function variants (pLI ~1.0), indicating that complete loss of protein function is likely not tolerated.

Summary from RefSeq
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0
Active trials
1
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.69
LOEUF
DN
Mechanism· predicted
Clinical SummaryLARP1B
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
0.69LOEUF
pLI 0.000
Z-score 3.41
OE 0.50 (0.370.69)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.52Z-score
OE missense 0.93 (0.861.01)
437 obs / 468.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.50 (0.370.69)
00.351.4
Missense OE0.93 (0.861.01)
00.61.4
Synonymous OE0.89
01.21.6
LoF obs/exp: 27 / 54.0Missense obs/exp: 437 / 468.8Syn Z: 1.04
DN
0.75top 25%
GOF
0.5660th %ile
LOF
0.3746th %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

LARP1B · protein map & ClinVar variants

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

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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