TSC2 (Tuberin) variants and mutations

TSC2 (also known as Tuberin) is a human protein-coding gene encoding a tuberin protein. Together with TSC1, it inactivates RHEB and restrains mTORC1 when growth conditions are unfavorable. Loss-of-function variants cause tuberous sclerosis complex with hamartomas and tumors affecting the brain, kidneys, skin, heart, lungs, and other organs. This analysis covers 7,629 TSC2 variants and mutations. Of these, 56% have computational variant effect predictions. Disease context includes tuberous sclerosis, tuberous sclerosis 2, and lymphangioleiomyomatosis. Example TSC2 variants include M1I, M1K, and M1T.

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 TSC2 variants

Examples include M1I, M1K, M1T, M1V, A2D, A2G, A2P, A2S. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.