DNAH7

Chr 2AR

dynein axonemal heavy chain 7

Also known as: CILD50

DNAH7 encodes a dynein heavy chain protein that generates force for respiratory cilia and sperm flagella beating through its ATPase activity. Biallelic mutations cause primary ciliary dyskinesia, an autosomal recessive disorder affecting respiratory function and fertility. This gene is not highly constrained against loss-of-function variants.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
GOFmechanismARLOEUF 0.841 OMIM phenotype
Clinical SummaryDNAH7
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Gene-Disease Validity (ClinGen)
primary ciliary dyskinesia · ARLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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ClinVar Variants
7 unique Pathogenic / Likely Pathogenic· 349 VUS of 395 total submissions
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GeneReview available — DNAH7
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

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 3.50
OE 0.73 (0.630.84)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-1.09Z-score
OE missense 1.07 (1.031.11)
2195 obs / 2055.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.73 (0.630.84)
00.351.4
Missense OE1.07 (1.031.11)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 140 / 192.4Missense obs/exp: 2195 / 2055.9Syn Z: -0.64
DN
0.5673th %ile
GOF
0.7028th %ile
LOF
0.3067th %ile

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction 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

395 submitted variants in ClinVar

Classification Summary

Pathogenic2
Likely Pathogenic5
VUS349
Likely Benign8
Benign1
Conflicting2
2
Pathogenic
5
Likely Pathogenic
349
VUS
8
Likely Benign
1
Benign
2
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
2
0
2
Likely Pathogenic
5
0
0
0
5
VUS
3
344
2
0
349
Likely Benign
0
6
0
2
8
Benign
0
1
0
0
1
Conflicting
2
Total835142367

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

View in ClinVar →

Protein Context — Lollipop Plot

DNAH7 · 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 →
Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 3 full-text resultsSearch PubTator3 ↗