Anti-SMN2 Antibody, clone SMN-KH

Survival of Motor Neuron (SMN) is expressed from two linked paralogous genes, SMN1 and SMN2. SMNs are primarily localized in the cytoplasm and nuclear gems of all cells, where they are understood to mediate the assembly of spliceosomal small nuclear ribonucleoprotein particles (snRNPs). They also play a role in the metabolism of snoRNPs and are required for the splicing of pre-mRNA in the nucleus. The progressive loss of functional SMNs in the anterior horn of the spinal cord is a critical cause of Spinal Muscular Atrophy (SMA), an autosomal recessive neuromuscular disease. There are three known types of childhood-onset SMA, in addition to a fourth type that is characterized by adult-onset SMA.

~35 kDa observed

MBP-tagged recombinant protein corresponding to human SMN2.

Anti-SMN2 Antibody, clone SMN-KH is a Mouse Monoclonal Antibody for detection of SMN2 also known as Survival motor neuron protein & has been validated in WB & IHC.

Western Blot Analysis: A representative lot from an independent laboratory detected SMN2 in HeLa cell lysate.

Immunohistochemistry Analysis: A respresentative lot from an independent laboratory detected human SMN2 in SMA type III mouse model thoracic spinal cord tissue. (Hua, Y., et al. (2010). Genes Dev. 24(15):1634-1644.)

Format: Purified

Evaluated by Western Blot in HeLa cell lysate.

Western Blot Analysis: 0.5 µg/mL of this antibody detected SMN2 in 10 µg of HeLa cell lysate.

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.