SERPINB7

Chr 18AR

serpin family B member 7

Also known as: MEGSIN, PPKN, TP55

This protein functions as a protease inhibitor that may specifically inhibit lysine-specific proteases and influence megakaryocyte maturation. Mutations cause palmoplantar keratoderma, Nagashima type, a condition affecting the skin of palms and soles, inherited in an autosomal recessive pattern. The gene shows low constraint to loss-of-function variation.

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

Palmoplantar keratoderma, Nagashima typeMIM #615598
AR
UniProtKeratoderma, palmoplantar, Nagashima type
0
Active trials
18
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.31
LOEUF
Multiple*
Mechanism· predicted
Clinical SummarySERPINB7
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
1.31LOEUF
pLI 0.000
Z-score 0.61
OE 0.84 (0.551.31)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.53Z-score
OE missense 1.10 (0.991.23)
225 obs / 203.6 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.84 (0.551.31)
00.351.4
Missense OE1.10 (0.991.23)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 14 / 16.7Missense obs/exp: 225 / 203.6Syn Z: -0.25
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveSERPINB7-related palmoplantar keratoderma, Nagashima typeOTHERAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.7228th %ile
GOF
0.6444th %ile
LOF
0.2288th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SERPINB7 · 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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