DCTPP1

Chr 16

dCTP pyrophosphatase 1

Also known as: CDA03, RS21C6, XTP3TPA

dCTP pyrophosphatase hydrolyzes deoxynucleoside triphosphates to monophosphates, with strong preference for dCTP and its analogs, likely protecting against incorporation of genotoxic nucleotide analogs during DNA synthesis. Mutations cause autosomal recessive developmental delay with seizures and intellectual disability. The gene shows moderate constraint against loss-of-function variants.

OMIMResearchSummary from RefSeq, UniProt
Multiplemechanism
Some data sources returned errors (1)

gnomad: TimeoutError: The operation was aborted due to timeout

Population Genetics & Constraint

Constraint data not available from gnomAD.

DN
0.7133th %ile
GOF
0.6735th %ile
LOF
0.2872th %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
GOFprediction 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.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DCTPP1 · 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 →