SOX2 (Transcription factor SOX-2) variants and mutations

SOX2 (also known as Transcription factor SOX-2) is a human protein-coding gene encoding a transcription factor SOX-2 protein. It maintains neural and embryonic progenitor identity and directs development of the eye, forebrain, pituitary, and other organs. Haploinsufficiency causes SOX2 disorder, frequently with anophthalmia or microphthalmia and variable neurodevelopmental, endocrine, and genital abnormalities. This analysis covers 1,217 SOX2 variants and mutations. Of these, 72% have computational variant effect predictions. Disease context includes anophthalmia/microphthalmia-esophageal atresia syndrome, Anophthalmia/microphthalmia - esophageal atresia, and neurodegenerative disease. Example SOX2 variants include Y2D, Y2N, and Y2H.

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 SOX2 variants

Examples include Y2D, Y2N, Y2H, Y2Y, N3K, p.Asn3del, M4I, M4K. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.