LARP7

Chr 4AR

La ribonucleoprotein 7, transcriptional regulator

Also known as: ALAZS, HDCMA18P, PIP7S, hLARP7

The LARP7 protein binds to specific small nuclear RNAs (7SK and U6 snRNAs) and regulates RNA polymerase II transcriptional elongation and mRNA splicing fidelity through its roles in ribonucleoprotein complexes. Biallelic mutations cause Alazami syndrome, an autosomal recessive disorder. This gene shows low constraint against loss-of-function variants (pLI near zero).

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.841 OMIM phenotype
Clinical SummaryLARP7
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
0.84LOEUF
pLI 0.000
Z-score 2.22
OE 0.55 (0.370.84)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.27Z-score
OE missense 1.04 (0.951.15)
297 obs / 284.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.55 (0.370.84)
00.351.4
Missense OE1.04 (0.951.15)
00.61.4
Synonymous OE1.27
01.21.6
LoF obs/exp: 16 / 28.9Missense obs/exp: 297 / 284.3Syn Z: -2.06
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveLARP7-related Alazami syndromeLOFAR

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

DN
0.6646th %ile
GOF
0.4777th %ile
LOF
0.4429th %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

LARP7 · 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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