SMAD4 (SMAD family member 4) variants and mutations

SMAD4 (also known as SMAD family member 4) is a human protein-coding gene encoding a SMAD family member 4 protein. It forms transcriptional complexes with activated receptor-regulated SMADs and is the central nuclear mediator shared by TGF-beta and BMP pathways. Germline loss-of-function variants cause juvenile polyposis or combined juvenile-polyposis-HHT, while specific gain-of-function variants cause Myhre syndrome. This analysis covers 2,847 SMAD4 variants and mutations. Of these, 38% have computational variant effect predictions. Disease context includes juvenile polyposis syndrome, juvenile polyposis/hereditary hemorrhagic telangiectasia syndrome, and Myhre syndrome. Example SMAD4 variants include M1?, M1I, and M1T.

Variant analysis overview

Variant and mutation evidence

Clinical, disease, and population context

Protein structure and variant hotspots

Data sources

Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, 3D Hotspot Analysis, Interaction Network Analysis, Protein Data Bank, AlphaFold DB, gnomAD constraint, Open Targets, ClinGen, MaveDB, LitVar.

Notable SMAD4 variants

Examples include M1?, M1I, M1T, M1V, D2A, D2E, D2H, D2N. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.