Mouse Monoclonal SMN1 Antibody

The survival motor neuron 1 (SMN1) gene encodes a critical protein involved in the assembly of small nuclear ribonucleoproteins (snRNPs), essential for pre-mRNA splicing. Loss of SMN1 function due to mutations or deletions causes spinal muscular atrophy (SMA), a neurodegenerative disorder characterized by motor neuron degeneration and muscle weakness. SMN1 antibodies are tools designed to detect and quantify the SMN protein in research and diagnostics. These antibodies are pivotal in studying SMN expression patterns, protein stability, and localization in cellular and animal models. Since SMN1 shares high homology with its paralog SMN2. distinguishing between their protein products can be challenging. However, some antibodies target unique epitopes or isoforms to differentiate SMN1-derived full-length SMN from SMN2-derived truncated forms. Commercial SMN1 antibodies are widely used in techniques like Western blotting, immunohistochemistry, and ELISA to assess SMN levels in SMA patient samples or therapeutic studies. Challenges include ensuring specificity, as cross-reactivity with SMN2 or degraded proteins may occur. Recent advancements in SMA therapies, such as antisense oligonucleotides (e.g., nusinersen) and gene replacement (e.g., onasemnogene abeparvovec), have driven demand for reliable SMN1 antibodies to monitor treatment efficacy and SMN restoration. Ongoing research focuses on optimizing antibody sensitivity for early SMA diagnosis and mechanistic studies of SMN function in neuronal health.