PTCH1 (Protein patched homolog 1) variants and mutations

PTCH1 (also known as Protein patched homolog 1) is a human protein-coding gene encoding a protein patched homolog 1 protein. It suppresses Smoothened in the absence of Hedgehog ligands and thereby keeps Hedgehog developmental signaling inactive until an appropriate signal is received. Germline loss-of-function variants cause Gorlin syndrome, while somatic pathway activation drives basal-cell carcinoma and other tumors. This analysis covers 5,949 PTCH1 variants and mutations. Of these, 49% have computational variant effect predictions. Disease context includes nevoid basal cell carcinoma syndrome, holoprosencephaly, and basal cell carcinoma. Example PTCH1 variants include A2V, S3A, and S3L.

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 PTCH1 variants

Examples include A2V, S3A, S3L, S3T, S3W, A4G, A4T, A4V. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.