BUB1B (O60566) variants and mutations

BUB1B (also known as O60566) is a human protein-coding gene encoding a mitotic checkpoint serine/threonine-protein kinase BUB1 beta protein. It enforces the spindle-assembly checkpoint and helps ensure that chromosomes are correctly attached before anaphase begins. Biallelic hypomorphic variants cause mosaic variegated aneuploidy syndrome, with growth abnormalities, developmental impairment, and elevated childhood cancer risk. This analysis covers 1,704 BUB1B variants and mutations. Of these, 69% have computational variant effect predictions. Disease context includes mosaic variegated aneuploidy syndrome, mosaic variegated aneuploidy syndrome 1, and premature chromatid separation trait. Example BUB1B variants include A2T, A2A, and A3T.

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 BUB1B variants

Examples include A2T, A2A, A3T, A3V, A3A, V4G, V4M, V4E. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.