LRRC56

Chr 11

leucine rich repeat containing 56

Also known as: CILD39, DNAAF12, oda8

The protein is required for the assembly of dynein arms in cilia and is predicted to be involved in cell projection organization. Mutations cause primary ciliary dyskinesia 39, an autosomal recessive disorder affecting the respiratory system and other ciliated organs. The gene is highly constrained against loss-of-function variants (pLI ~1.0), indicating that complete loss of function is likely not tolerated.

GeneReviewsResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 1.25
Clinical SummaryLRRC56
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Gene-Disease Validity (ClinGen)
ciliary dyskinesia, primary, 39 · ARModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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ClinVar Variants
11 unique Pathogenic / Likely Pathogenic· 75 VUS of 200 total submissions
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GeneReview available — LRRC56
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.25LOEUF
pLI 0.000
Z-score 0.74
OE 0.82 (0.551.25)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.88Z-score
OE missense 1.14 (1.041.25)
353 obs / 309.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.82 (0.551.25)
00.351.4
Missense OE1.14 (1.041.25)
00.61.4
Synonymous OE1.37
01.21.6
LoF obs/exp: 16 / 19.5Missense obs/exp: 353 / 309.3Syn Z: -3.38
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongLRRC56-related mucociliary clearance and laterality defectsLOFAR

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

DN
0.5967th %ile
GOF
0.72top 25%
LOF
0.4233th %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. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

200 submitted variants in ClinVar

Classification Summary

Pathogenic3
Likely Pathogenic8
VUS75
Likely Benign100
Benign8
Conflicting2
3
Pathogenic
8
Likely Pathogenic
75
VUS
100
Likely Benign
8
Benign
2
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
2
0
1
0
3
Likely Pathogenic
2
2
4
0
8
VUS
7
59
9
0
75
Likely Benign
1
5
44
50
100
Benign
0
0
8
0
8
Conflicting
2
Total12666650196

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

View in ClinVar →

Protein Context — Lollipop Plot

LRRC56 · 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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