RAG2 (P55895) variants and mutations

RAG2 (also known as P55895) is a human protein-coding gene encoding a v(D)J recombination-activating protein 2 protein. Together with RAG1, it restricts and activates V(D)J recombination during lymphocyte development so immunoglobulin and T-cell receptor genes can be assembled. Biallelic loss-of-function variants cause severe combined immunodeficiency or hypomorphic immune-dysregulation syndromes. This analysis covers 1,330 RAG2 variants and mutations. Of these, 74% have computational variant effect predictions. Disease context includes severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-n, combined immunodeficiency with skin granulomas, and Omenn syndrome. Example RAG2 variants include M1?, M1T, and M1V.

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 RAG2 variants

Examples include M1?, M1T, M1V, S2C, Q4H, M5K, M5L, M5R. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.