TBK1 (Q9UHD2) variants and mutations

TBK1 (also known as Q9UHD2) is a human protein-coding gene encoding a serine/threonine-protein kinase protein. It activates IRF3 and related antiviral pathways after innate immune sensing and also participates in autophagy and cellular homeostasis. Loss-of-function variants can cause amyotrophic lateral sclerosis or frontotemporal dementia, while increased pathway activity can support inflammatory disease and some cancers. This analysis covers 901 TBK1 variants and mutations. Of these, 76% have computational variant effect predictions. Disease context includes frontotemporal dementia and/or amyotrophic lateral sclerosis 4, Herpetic encephalitis, and amyotrophic lateral sclerosis. Example TBK1 variants include M1T, Q2*, and Q2H.

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, MaveDB, LitVar.

Notable TBK1 variants

Examples include M1T, Q2*, Q2H, Q2R, S3N, S3S, T4S, S5C. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.