LRRC47

Chr 1

leucine rich repeat containing 47

The protein enables RNA binding and is predicted to be involved in phenylalanyl-tRNA aminoacylation, a process essential for protein synthesis. Mutations cause autosomal recessive intellectual developmental disorder with seizures and language delay. This gene is highly constrained against loss-of-function variants (pLI 0.87, LOEUF 0.41), indicating that complete loss of function is likely not tolerated.

Summary from RefSeq
Research Assistant →
0
Active trials
0
Pubs (1 yr)
92
P/LP submissions
0%
P/LP missense
0.41
LOEUF
Mechanism
Clinical SummaryLRRC47
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.87) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
87 unique Pathogenic / Likely Pathogenic· 88 VUS of 193 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.41LOEUF
pLI 0.871
Z-score 3.17
OE 0.13 (0.050.41)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.03Z-score
OE missense 0.84 (0.760.93)
263 obs / 314.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.13 (0.050.41)
00.351.4
Missense OE0.84 (0.760.93)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 2 / 15.5Missense obs/exp: 263 / 314.2Syn Z: -0.71

ClinVar Variant Classifications

193 submitted variants in ClinVar

Classification Summary

Pathogenic85
Likely Pathogenic2
VUS88
Likely Benign1
Benign1
85
Pathogenic
2
Likely Pathogenic
88
VUS
1
Likely Benign
1
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
85
0
85
Likely Pathogenic
0
0
2
0
2
VUS
0
76
12
0
88
Likely Benign
0
1
0
0
1
Benign
0
0
1
0
1
Total0771000177

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

View in ClinVar →

Protein Context — Lollipop Plot

LRRC47 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found