P2RY12

Chr 3AR

purinergic receptor P2Y12

This gene encodes a G-protein coupled receptor for ADP and ATP that inhibits adenylyl cyclase and is required for normal platelet aggregation and blood coagulation. Mutations cause bleeding disorder, platelet-type 8, an autosomal recessive condition affecting hemostasis. The gene is not highly constrained against loss-of-function variants (pLI = 0.002, LOEUF = 1.41), consistent with its recessive inheritance pattern.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismARLOEUF 1.411 OMIM phenotype
Clinical SummaryP2RY12
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Gene-Disease Validity (ClinGen)
platelet-type bleeding disorder 8 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

2 total gene-disease associations curated

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.41LOEUF
pLI 0.002
Z-score 0.82
OE 0.68 (0.351.41)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.86Z-score
OE missense 0.82 (0.720.94)
152 obs / 185.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.68 (0.351.41)
00.351.4
Missense OE0.82 (0.720.94)
00.61.4
Synonymous OE0.97
01.21.6
LoF obs/exp: 5 / 7.4Missense obs/exp: 152 / 185.0Syn Z: 0.18
DN
0.78top 25%
GOF
0.80top 10%
LOF
0.2288th %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
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.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

P2RY12 · 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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