DRC1

Chr 2AR

dynein regulatory complex subunit 1

Also known as: C2orf39, CCDC164, CILD21, SPGF80

The DRC1 protein is a central component of the nexin-dynein complex that regulates ciliary dynein assembly, essential for proper ciliary function. Mutations cause autosomal recessive primary ciliary dyskinesia and male infertility due to spermatogenic failure. This gene is not highly constrained against loss-of-function variants, consistent with its recessive inheritance pattern affecting ciliary motility in respiratory tract and reproductive systems.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.042 OMIM phenotypes
Clinical SummaryDRC1
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Gene-Disease Validity (ClinGen)
primary ciliary dyskinesia 21 · 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
1.04LOEUF
pLI 0.000
Z-score 1.33
OE 0.79 (0.601.04)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.05Z-score
OE missense 1.01 (0.931.09)
402 obs / 399.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.79 (0.601.04)
00.351.4
Missense OE1.01 (0.931.09)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 35 / 44.6Missense obs/exp: 402 / 399.4Syn Z: -0.48
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongDRC1-related primary ciliary dyskinesiaLOFAR

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

DN
0.75top 25%
GOF
0.6150th %ile
LOF
0.2969th %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.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DRC1 · 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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