CST, 12976T, SMN1 (2F1) Mouse Monoclonal Antibody

Monoclonal Antibody for studying SMN1. Validated for Western Blotting,Immunoprecipitation,Immunofluorescence (Immunocytochemistry). Available in 2 sizes. Highly specific and rigorously validated in-house, SMN1 (2F1) Mouse Monoclonal Antibody (CST #12976) is ready to ship. Product Usage Information Western Blotting: 1:1000 Immunoprecipitation: 1:50 Immunofluorescence (Immunocytochemistry): 1:400 Storage Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody. Protocol Available protocols: Western Blotting, Immunoprecipitation, Immunofluorescence (Immunocytochemistry) Specificity / Sensitivity SMN1 (2F1) Mouse Monoclonal Antibody recognizes endogenous levels of total SMN1 protein. Species Reactivity: Human, Monkey Source / Purification Monoclonal antibody is produced by immunizing animals with recombinant protein specific to the carboxy terminus of human SMN1 protein. Background The survival motor neuron (SMN) protein is ubiquitously expressed in mammals and encoded by two genes, and (1,2). The protein coding sequences of SMN1 and SMN2 are predicted to be identical, as SMN2 differs from SMN1 by only five nucleotides (3). SMN impacts various aspects of RNA metabolism, and is required for biogenesis of small nuclear ribonucleoprotein (snRNP) particles critical for pre-mRNA splicing. Furthermore, SMN, needed for stress granule formation, is found in ribonucleoprotein (RNP) granules moving through neuronal processes and is part of RNP complexes implicated in synaptic local translation (4,5). Mutations in its coding genes, or , are linked to neuromuscular disease spinal muscular atrophy (SMA), a leading genetic cause of infant mortality (2-6). The severity of SMA is inversely proportional to SMN2 copy number. Over 96% of SMA patients have homozygous mutations (deletion, rearrangement, or point mutation) in SMN1, however they retain at least one copy of SMN2 (1). Alternate Names BCD541; Component of gems 1; gemin 1; Gemin-1; GEMIN1; SMA; SMA@; SMA1; SMA2; SMA3; SMA4; SMN; SMN1; SMN2; SMNC; SMNT; survival motor neuron 1 protein; Survival motor neuron protein; survival of motor neuron 1, telomeric; T-BCD541; TDRD16A; tudor domain containing 16A Specification REACTIVITY: H Mk SENSITIVITY: Endogenous MW (kDa): 38 Source/Isotype: Mouse IgG1