LRP2 (P98164) variants and mutations

LRP2 (also known as P98164) is a human protein-coding gene encoding a low-density lipoprotein receptor-related protein 2 protein. It mediates endocytic uptake of filtered proteins, vitamins, hormones, and other ligands in proximal renal tubules and several absorptive epithelia. Biallelic loss-of-function variants cause Donnai-Barrow syndrome with proteinuria, craniofacial abnormalities, and neurodevelopmental features. This analysis covers 5,813 LRP2 variants and mutations. Of these, 70% have computational variant effect predictions. Disease context includes Donnai-Barrow syndrome, Intellectual disability, and gout. Example LRP2 variants include R3C, R3H, and R3P.

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, gnomAD constraint, Open Targets, ClinGen, MaveDB, LitVar.

Notable LRP2 variants

Examples include R3C, R3H, R3P, G4R, G4W, P5L, P5R, A6E. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.