SMN1 (Survival motor neuron protein) variants and mutations

SMN1 (also known as Survival motor neuron protein) is a human protein-coding gene encoding a survival motor neuron protein. It is required for assembly of small nuclear ribonucleoproteins and other RNA-protein complexes, with motor neurons being especially sensitive to reduced protein levels. Biallelic loss of functional SMN1 causes spinal muscular atrophy, whose severity is strongly modified by SMN2 copy number. This analysis covers 244 SMN1 variants and mutations. Of these, 88% have computational variant effect predictions. Disease context includes Proximal spinal muscular atrophy type 3, spinal muscular atrophy, type 1, and spinal muscular atrophy, type III. Example SMN1 variants include A2G, A2V, and A2A.

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, AlphaFold DB, gnomAD constraint, Open Targets, MaveDB, LitVar.

Notable SMN1 variants

Examples include A2G, A2V, A2A, M3E, M3V, M3L, M3K, M3R. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.