PDE3A

Chr 12AD

phosphodiesterase 3A

Also known as: CGI-PDE, CGI-PDE A, CGI-PDE-A, HTNB

The protein is a cyclic nucleotide phosphodiesterase that hydrolyzes cAMP and cGMP to regulate intracellular signaling, mediating platelet aggregation and controlling vascular smooth muscle contraction and relaxation. Mutations cause hypertension and brachydactyly syndrome with autosomal dominant inheritance. The gene is highly constrained against loss-of-function variants (pLI near 0, LOEUF 0.515), suggesting intolerance to protein-disrupting mutations.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

Hypertension and brachydactyly syndromeMIM #112410
AD
0
Active trials
25
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.52
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryPDE3A
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.33) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.52LOEUF
pLI 0.000
Z-score 4.13
OE 0.33 (0.220.52)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.59Z-score
OE missense 0.93 (0.871.00)
586 obs / 627.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.33 (0.220.52)
00.351.4
Missense OE0.93 (0.871.00)
00.61.4
Synonymous OE1.16
01.21.6
LoF obs/exp: 15 / 44.8Missense obs/exp: 586 / 627.8Syn Z: -2.00
DN
0.6453th %ile
GOF
0.6345th %ile
LOF
0.4038th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOF1 literature citation

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 mutations increased protein kinase A-mediated PDE3A phosphorylation and resulted in gain of function, with increased cAMP-hydrolytic activity and enhanced cell proliferation.PMID:25961942

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PDE3A · 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.

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Clinical Literature
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