DNAH11

Chr 7AR

dynein axonemal heavy chain 11

Also known as: CILD7, DNAHBL, DNAHC11, DNHBL, DPL11

This gene encodes a ciliary outer dynein arm protein that generates force for cilia beating in respiratory epithelia and is essential for structural and functional integrity of cilia. Mutations cause primary ciliary dyskinesia, which may present with or without situs inversus and affects respiratory function due to impaired ciliary motility. The condition follows autosomal recessive inheritance.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismARLOEUF 0.671 OMIM phenotype
Clinical SummaryDNAH11
🧬
Gene-Disease Validity (ClinGen)
primary ciliary dyskinesia 7 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

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
0.67LOEUF
pLI 0.000
Z-score 5.79
OE 0.58 (0.510.67)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-5.61Z-score
OE missense 1.33 (1.291.37)
3027 obs / 2274.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.58 (0.510.67)
00.351.4
Missense OE1.33 (1.291.37)
00.61.4
Synonymous OE1.28
01.21.6
LoF obs/exp: 131 / 224.6Missense obs/exp: 3027 / 2274.4Syn Z: -6.34
DN
0.6551th %ile
GOF
0.73top 25%
LOF
0.2093th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
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

DNAH11 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →