F10

Chr 13AR

coagulation factor X

Also known as: FX, FXA

Factor X is a vitamin K-dependent serine protease that converts prothrombin to thrombin during blood coagulation and activates pro-inflammatory signaling pathways through protease-activated receptors. Mutations cause factor X deficiency, an autosomal recessive bleeding disorder with variable severity ranging from mild bruising to life-threatening hemorrhage. The gene shows very low constraint against loss-of-function variants (pLI near 0), consistent with recessive inheritance where heterozygous carriers are typically unaffected.

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

Factor X deficiencyMIM #227600
AR
0
Active trials
319
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.97
LOEUF
Multiple*
Mechanism· predicted
Clinical SummaryF10
🧬
Gene-Disease Validity (ClinGen)
congenital factor X deficiency · 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
0.97LOEUF
pLI 0.000
Z-score 1.67
OE 0.60 (0.380.97)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.20Z-score
OE missense 0.81 (0.730.90)
245 obs / 303.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.60 (0.380.97)
00.351.4
Missense OE0.81 (0.730.90)
00.61.4
Synonymous OE0.95
01.21.6
LoF obs/exp: 12 / 20.0Missense obs/exp: 245 / 303.7Syn Z: 0.46
DN
0.7035th %ile
GOF
0.7028th %ile
LOF
0.2775th %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

F10 · 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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