Anti-SMN Antibody, clone 62E7

产品说明

一般描述

The survival of motor neurons (SMN) protein is essential for the biogenesis of small nuclear RNA (snRNA)-ribonucleoproteins (snRNPs), the major components of the pre-mRNA splicing machinery. Though it is ubiquitously expressed, SMN deficiency causes the motor neuron degenerative disease spinal muscular atrophy (SMA). SMN deficiency, similar to that which occurs in severe SMA, has unexpected cell type-specific effects on the repertoire of snRNAs and mRNAs. It alters the stoichiometry of snRNAs and causes widespread pre-mRNA splicing defects in numerous transcripts of diverse genes, preferentially those containing a large number of introns, in SMN-deficient mouse tissues. These findings reveal a key role for the SMN complex in RNA metabolism and in splicing regulation and indicate that SMA is a general splicing disease that is not restricted to motor neurons.

特异性

Recognizes SMN.

免疫原

His-tagged recombinant protein corresponding to human SMN.
Epitope: Unknown

应用

Research Category
Epigenetics & Nuclear Function
Use Anti-SMN Antibody, clone 62E7 (Mouse Monoclonal Antibody) validated in WB, ICC, IP to detect SMN also known as Spinal muscular atrophy (Werdnig-Hoffmann disease Kugelberg-Welander disease).
Immunocytochemistry Analysis: 1:500 dilution from a representative lot detected SMN in HeLa cells.
Research Sub Category
RNA Metabolism & Binding Proteins

质量

Evaluated by Western Blot in HeLa cell lysate.
Western Blot Analysis: 0.5 µg/ml of this antibody detected SMN on 10 µg of HeLa cell lysate.

目标描述

35 kDa was observed; however, the calculated molecular weight is 31.849 kDa

外形

Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.05% sodium azide.
Format: Purified
Protein G Purified

储存及稳定性

Stable for 1 year at 2-8°C from date of receipt.

分析说明

Control
HeLa cell lysate

其他说明

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

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

基本信息

产品性质

安全信息