SLC26A4 (Pendrin) variants and mutations

SLC26A4 (also known as Pendrin) is a human protein-coding gene encoding a pendrin protein. SLC26A4, known as pendrin, is an anion exchanger that transports chloride, iodide, and bicarbonate without directly using sodium. It supports ion balance in the inner ear and thyroid, and SLC26A4 variants cause Pendred syndrome and inherited deafness. This analysis covers 1,712 SLC26A4 variants and mutations. Of these, 97% have computational variant effect predictions. Disease context includes Pendred syndrome, autosomal recessive nonsyndromic hearing loss 4, and hearing loss, autosomal recessive. Example SLC26A4 variants include M1?, M1I, and M1R.

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, AlphaFold DB, gnomAD constraint, Open Targets, PharmGKB, MaveDB, LitVar.

Notable SLC26A4 variants

Examples include M1?, M1I, M1R, M1T, A2V, A2T, A2S, A2E. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.