SERPINE2

Chr 2

serpin family E member 2

Also known as: GDN, GDNPF, PI-7, PI7, PN-1, PN1, PNI

SERPINE2 encodes a serine protease inhibitor that inhibits thrombin, trypsin, and urokinase, and promotes neurite extension by inhibiting thrombin. Mutations cause autosomal recessive progressive myoclonic epilepsy, with seizures and progressive neurological decline typically beginning in childhood or adolescence. This gene is highly constrained against loss-of-function variants, indicating that such mutations are likely to be pathogenic.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
43
Pubs (1 yr)
30
P/LP submissions
0%
P/LP missense
0.45
LOEUF
Mechanism
Clinical SummarySERPINE2
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.73) — some intolerance to loss-of-function variants.
📋
ClinVar Variants
30 unique Pathogenic / Likely Pathogenic· 46 VUS of 91 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.45LOEUF
pLI 0.730
Z-score 3.20
OE 0.17 (0.080.45)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.59Z-score
OE missense 0.71 (0.630.81)
170 obs / 239.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.17 (0.080.45)
00.351.4
Missense OE0.71 (0.630.81)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 3 / 17.4Missense obs/exp: 170 / 239.1Syn Z: -0.39

ClinVar Variant Classifications

91 submitted variants in ClinVar

Classification Summary

Pathogenic29
Likely Pathogenic1
VUS46
Likely Benign4
29
Pathogenic
1
Likely Pathogenic
46
VUS
4
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
29
0
29
Likely Pathogenic
0
0
1
0
1
VUS
0
43
3
0
46
Likely Benign
0
3
0
1
4
Benign
0
0
0
0
0
Total04633180

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

SERPINE2 · 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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