F11

Chr 4

coagulation factor XI

Also known as: FXI, PTA

This gene encodes coagulation factor XI of the blood coagulation cascade. This protein is present in plasma as a zymogen, which is a unique plasma coagulation enzyme because it exists as a homodimer consisting of two identical polypeptide chains linked by disulfide bonds. During activation of the plasma factor XI, an internal peptide bond is cleaved by factor XIIa (or XII) in each of the two chains, resulting in activated factor XIa, a serine protease composed of two heavy and two light chains held together by disulfide bonds. This activated plasma factor XI triggers the middle phase of the intrisic pathway of blood coagulation by activating factor IX. Defects in this factor lead to Rosenthal syndrome, a blood coagulation abnormality. [provided by RefSeq, Jul 2008]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Factor XI deficiency, autosomal dominantMIM #612416
Factor XI deficiency, autosomal recessiveMIM #612416

Clinical highlights

Gene-disease validity (ClinGen)
congenital factor XI deficiency · SDDefinitivesufficient evidence for diagnostic panels
0
Active trials
126
Pubs (1 yr)
P/LP submissions
P/LP missense
1.45
LOEUF
Multiple*
Mechanism· predicted
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.45LOEUF
pLI 0.000
Z-score -0.53
OE 1.10 (0.841.45)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.02Z-score
OE missense 1.00 (0.911.09)
342 obs / 343.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?1.10 (0.841.45)
00.351.4
Missense OE?1.00 (0.911.09)
00.61.4
Synonymous OE?1.05
01.21.6
LoF obs/exp: 37 / 33.7Missense obs/exp: 342 / 343.0Syn Z: -0.49

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

F11 · 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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