STAMBP

Chr 2AR

STAM binding protein

Also known as: AMSH, MICCAP

STAMBP encodes a zinc metalloprotease that cleaves specific polyubiquitin chains and regulates endosomal sorting of cell surface receptors to lysosomes, playing key roles in cytokine signaling, BMP signaling, and receptor-mediated endocytosis. Autosomal recessive mutations cause microcephaly-capillary malformation syndrome, affecting brain development and vascular formation. The gene shows low constraint to loss-of-function variation (pLI 0.00005), consistent with its recessive inheritance pattern.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.761 OMIM phenotype
Clinical SummarySTAMBP
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
0.76LOEUF
pLI 0.000
Z-score 2.43
OE 0.46 (0.290.76)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.89Z-score
OE missense 0.84 (0.750.94)
202 obs / 240.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.46 (0.290.76)
00.351.4
Missense OE0.84 (0.750.94)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 11 / 23.8Missense obs/exp: 202 / 240.7Syn Z: -0.03
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveSTAMBP-related microcephaly-capillary malformation syndromeLOFAR

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

DN
0.6745th %ile
GOF
0.5758th %ile
LOF
0.2968th %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

STAMBP · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

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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