DNAAF19

Chr 17AR

dynein axonemal assembly factor 19

Also known as: CCDC103, CILD17, PR46b, SMH

The protein is a dynein-attachment factor that enables protein homodimerization and is required for axonemal dynein complex assembly and cilia motility. Biallelic mutations cause primary ciliary dyskinesia 17, an autosomal recessive disorder resulting from defective outer dynein arm function leading to impaired cilium movement and disrupted left-right body asymmetry.

Summary from RefSeq, OMIM, UniProt

Primary Disease Associations & Inheritance

Ciliary dyskinesia, primary, 17MIM #614679
AR
0
Active trials
0
Pubs (1 yr)
75
P/LP submissions
5%
P/LP missense
LOEUF
LOF
Mechanism· G2P
Clinical SummaryDNAAF19
🧬
Gene-Disease Validity (ClinGen)
primary ciliary dyskinesia 17 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

📋
ClinVar Variants
37 unique Pathogenic / Likely Pathogenic· 81 VUS of 218 total submissions
📖
GeneReview available — DNAAF19
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

218 submitted variants in ClinVar

Classification Summary

Pathogenic23
Likely Pathogenic14
VUS81
Likely Benign79
Benign8
Conflicting9
23
Pathogenic
14
Likely Pathogenic
81
VUS
79
Likely Benign
8
Benign
9
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
5
0
18
0
23
Likely Pathogenic
9
2
3
0
14
VUS
1
55
24
1
81
Likely Benign
1
9
16
53
79
Benign
0
1
7
0
8
Conflicting
9
Total16676854214

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

View in ClinVar →

Protein Context — Lollipop Plot

DNAAF19 · 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 →
Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 2 full-text resultsSearch PubTator3 ↗
Key Publications
Landmark & review papers · by relevance
PubMed
Primary Ciliary Dyskinesia.
Adam MP et al.
2025Review
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found