LRRC41

Chr 1

leucine rich repeat containing 41

Also known as: MUF1, PP7759

The protein functions as a substrate recognition component of an E3 ubiquitin ligase complex that targets proteins for degradation via the ubiquitin-proteasome system. Mutations cause autosomal recessive intellectual disability with seizures and developmental delay, typically presenting in early childhood. This gene is highly constrained against loss-of-function variants in the general population.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.18
Clinical SummaryLRRC41
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.18LOEUF
pLI 1.000
Z-score 5.09
OE 0.06 (0.020.18)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
3.08Z-score
OE missense 0.61 (0.550.67)
300 obs / 492.4 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.06 (0.020.18)
00.351.4
Missense OE0.61 (0.550.67)
00.61.4
Synonymous OE0.92
01.21.6
LoF obs/exp: 2 / 34.1Missense obs/exp: 300 / 492.4Syn Z: 0.87
DN
0.2199th %ile
GOF
0.2995th %ile
LOF
0.72top 10%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.18

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

LRRC41 · 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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