LRBA (P50851) variants and mutations

LRBA (also known as P50851) is a human protein-coding gene encoding a lipopolysaccharide-responsive and beige-like anchor protein. It regulates intracellular trafficking of immune proteins, including recycling and preservation of CTLA-4 in regulatory T cells. Biallelic loss-of-function variants cause immune dysregulation with autoimmunity, lymphoproliferation, recurrent infection, and inflammatory bowel disease. This analysis covers 3,609 LRBA variants and mutations. Of these, 64% have computational variant effect predictions. Disease context includes combined immunodeficiency due to LRBA deficiency, colobomatous microphthalmia-rhizomelic dysplasia syndrome, and cholelithiasis. Example LRBA variants include M1V, S3N, and S3R.

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, Interaction Network Analysis, Protein Data Bank, gnomAD constraint, Open Targets, ClinGen, MaveDB, LitVar.

Notable LRBA variants

Examples include M1V, S3N, S3R, E4K, D5N, N6D, N6K, N6S. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.