SMN (survival motor neuron) was discovered as a candidate gene, located in chromosome 5q13, for the fatal autosomal Spinal muscular
atrophy (SMA) disorder. The SMN gene was missing or interrupted in a significant number of patients with SMA. The SMN protein is 294
amino acids and migrates with apparent molecular weight of 40 kDa. In addition to the cytoplasm, other studies localized SMN in dots of
0.1-1.0 μm within the nucleus. These novel nuclear structures were named "gems" and found associated to coiled bodies. It was also found
that SMN interacts with the RGG box of hnRNP U and fibrillarin. Therefore, the biochemical function of SMN may be in the regulation of
mRNA metabolism.
原厂资料:
注意事项:
1.Since applications vary, each investigator should titrate the reagent to obtain optimal results.
2.Caution: Sodium azide yields highly toxic hydrazoic acid under acidic conditions. Dilute azide compounds in running water before discarding to avoid accumulation of potentially explosive deposits in plumbing.