DRD3

Chr 3AD

dopamine receptor D3

Also known as: D3DR, ETM1, FET1

The DRD3 protein encodes a dopamine receptor primarily expressed in limbic brain areas that inhibits adenylyl cyclase via G protein coupling and regulates cognitive, emotional, and endocrine functions. Mutations cause autosomal dominant hereditary essential tremor type 1, with genetic variants also associated with increased susceptibility to schizophrenia. This gene shows low constraint against loss-of-function variants (pLI 0.002), suggesting that complete loss of function may be tolerated.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismADLOEUF 0.812 OMIM phenotypes
Clinical SummaryDRD3
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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ClinVar Variants
32 unique Pathogenic / Likely Pathogenic· 47 VUS of 107 total submissions
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Clinical Trials
3 active or recruiting trials — potential therapeutic options may be available
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GeneReview available — DRD3
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
0.81LOEUF
pLI 0.002
Z-score 2.13
OE 0.43 (0.240.81)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.47Z-score
OE missense 0.73 (0.640.83)
169 obs / 232.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.43 (0.240.81)
00.351.4
Missense OE0.73 (0.640.83)
00.61.4
Synonymous OE1.01
01.21.6
LoF obs/exp: 7 / 16.3Missense obs/exp: 169 / 232.0Syn Z: -0.09
DN
0.80top 10%
GOF
0.82top 10%
LOF
0.2580th %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 · 1 literature citation
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.

Literature Evidence

GOFThe gain-of-function produced by the Gly-9 variant may explain why drugs active against tremor in Parkinson's disease (PD) are usually not effective in the treatment of ET and suggests that DRD3 partial agonists or antagonists should be considered as novel therapeutic options for patients with ET.PMID:16809426

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

107 submitted variants in ClinVar

Classification Summary

Pathogenic31
Likely Pathogenic1
VUS47
Likely Benign16
Benign7
Conflicting2
31
Pathogenic
1
Likely Pathogenic
47
VUS
16
Likely Benign
7
Benign
2
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
31
0
31
Likely Pathogenic
0
0
1
0
1
VUS
1
42
4
0
47
Likely Benign
0
3
4
9
16
Benign
0
0
3
4
7
Conflicting
2
Total1454313104

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

View in ClinVar →

Protein Context — Lollipop Plot

DRD3 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
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