SERPIND1

Chr 22AD

serpin family D member 1

Also known as: D22S673, HC2, HCF2, HCII, HLS2, LS2, THPH10

This gene belongs to the serpin gene superfamily. Serpins play roles in many processes including inflammation, blood clotting, and cancer metastasis. Members of this family have highly conserved secondary structures with a reactive center loop that interacts with the protease active site to inhibit protease activity. This gene encodes a plasma serine protease that functions as a thrombin and chymotrypsin inhibitor. The protein is activated by heparin, dermatan sulfate, and glycosaminoglycans. Allelic variations in this gene are associated with heparin cofactor II deficiency. [provided by RefSeq, Jul 2015]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Thrombophilia 10 due to heparin cofactor II deficiencyMIM #612356
AD
Thrombophilia 10 due to heparin cofactor II deficiencyMIM #612356
AD
UniProtThrombophilia due to heparin cofactor 2 deficiency

Clinical highlights

Gene-disease validity (ClinGen)
heparin cofactor 2 deficiency · ADDefinitivesufficient evidence for diagnostic panels
0
Active trials
17
Pubs (1 yr)
P/LP submissions
P/LP missense
1.50
LOEUF
Multiple*
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.50LOEUF
pLI 0.000
Z-score 0.09
OE 0.97 (0.651.50)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.06Z-score
OE missense 1.01 (0.921.11)
288 obs / 285.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.97 (0.651.50)
00.351.4
Missense OE?1.01 (0.921.11)
00.61.4
Synonymous OE?1.11
01.21.6
LoF obs/exp: 15 / 15.4Missense obs/exp: 288 / 285.3Syn Z: -0.90

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SERPIND1 · 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.

Search ClinicalTrials.gov →