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DRC4

Chr 16AR

dynein regulatory complex subunit 4

Also known as: CILD33, GAS11, GAS8

The DRC4 protein is a component of the nexin-dynein regulatory complex that regulates ciliary and flagellar motility and maintains axonemal integrity in both motile and primary cilia. Mutations cause primary ciliary dyskinesia, a condition affecting respiratory cilia function and other ciliated organs. Inheritance is autosomal recessive.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismAR2 OMIM phenotypes
Clinical SummaryDRC4
🧬
Gene-Disease Validity (ClinGen)
primary ciliary dyskinesia 33 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

📋
ClinVar Variants
45 unique Pathogenic / Likely Pathogenic· 82 VUS of 270 total submissions
📖
GeneReview available — DRC4
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/DRC4?content-type=application/json&expand=1

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

270 submitted variants in ClinVar

Classification Summary

Pathogenic36
Likely Pathogenic9
VUS82
Likely Benign96
Benign33
Conflicting3
36
Pathogenic
9
Likely Pathogenic
82
VUS
96
Likely Benign
33
Benign
3
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
7
0
29
0
36
Likely Pathogenic
4
0
5
0
9
VUS
0
63
18
1
82
Likely Benign
0
5
51
40
96
Benign
0
4
25
4
33
Conflicting
3
Total117212845259

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

View in ClinVar →

Protein Context — Lollipop Plot

DRC4 · 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 →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC