Success for our human SMN2 monoclonal antibody in recent validation experiments
admin March 7, 2011
Print 
PDF
Western blot with SMN2 Antibody (60154-1-Ig) with Hep2g cell lysate
If you are looking for an antibody that is highly specific for human SMN2 for your research, our SMN2 monoclonal antibody (60154-1-Ig) might be suitable for your requirements. A very recent peptide blocking experiment, done by our Proteintech researchers, has shown this particular antibody can be successfully neutralized by an excess of antigen peptide; therefore ruling out any non-specific binding. Furthermore, this particular SMN2 antibody is raised against amino acids 171-189 of human SMN2 and, as this latest validation experiment also confirms, it does not cross-react with mouse SMN2. SMN2 is particularly significant for research concerning SMA, an autosomal recessive neurodegenerative disease.
SMN2: a causative and prognostic factor in SMA cases
Survival of motor neuron (SMN) genes function in snRNP assembly in all cell types and are clearly vital to embryonic development, as lack of endogenous SMN in mice results in embryonic lethality. Two forms of the SMN gene exist in humans, SMN1 and SMN2. Both genes reside in the same genomic region and are 99.9% identical – with one amino acid exception: SMN2 has a C to T base transition that causes more than 80% of SMN2 transcript to lack exon 7 (SMNΔ7).
The majority of evidence suggests the resulting SMNΔ7 protein is not fully functional, is highly unstable and rapidly degraded. Normally, the presence of the SMN1 gene counteracts the loss of SMN protein through faulty SMN2 transcription; however, homozygous loss of the SMN1 gene results in the SMA disease phenotype – characterized by degeneration of the motor neurons of the spinal cord and atrophy of the muscles due to denervation. The disease has varying degrees of onset and severity, ranging from premature death due to breathing difficulties in infanthood to somewhat manageable symptoms in older children and young adults – with a large spectrum in between. The copy number of SMN2 genes present appears to predict the severity of each SMA case; more SMN2 copies means there is a greater chance of functional SMN2 protein expression and compensation for the loss of SMN1. In summary, increasing SMN2 copy numbers ultimately correlate with milder symptoms and a later onset age of SMA. It is thought that detecting SMN2 protein may be a successful diagnostic tool for the future prognoses of SMA patients.
IHC of paraffin-embedded Human brain using SMN2 antibody – 60154-1-Ig (10x objective)
If you are looking for an antibody that is highly specific for human SMN2 for your research, our SMN2 monoclonal antibody (60154-1-Ig) might be suitable for your requirements. A very recent peptide blocking experiment, done by our Proteintech researchers, has shown this particular antibody can be successfully neutralized by an excess of antigen peptide; therefore ruling out any non-specific binding. Furthermore, this particular SMN2 antibody is raised against amino acids 171-189 of human SMN2 and, as this latest validation experiment also confirms, it does not cross-react with mouse SMN2. SMN2 is particularly significant for research concerning SMA, an autosomal recessive neurodegenerative disease.