Anticorps Monoclonal Anti - SMN produit dans la souris, environ 2 mg/ mL, clone 2B1, immunoglobulin purifié, solution aqueuse tamponnée

Non disponible en dehors du Royaume-Uni et de l'Irlande

Application

Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.Enzyme-linked immunosorbent assay (1 paper)

Mouse monoclonal clone 2B1 anti-SMN antibody is an important tool for studying the role of the survival of motor neurons protein in nuclear processes and spinal muscular atrophy (SMA). It may be used in immunoblotting (~35 kDa), immunoprecipitation, and immunocytochemistry.

Biochem/physiol Actions

Survival of Motor Neurons (SMN) complex is important in various biological processes, such as assembly and restructuring of spliceosomal small nuclear ribonucleoproteins (snRNPs), pre mRNA splicing and transcription. Spinal muscular atrophy (SMA) is caused by reduced expression or mutations in the survival of motor neurons (SMN) protein. Deletion or mutation in the telomeric copy (SMN1) causes the SMA phenotype. The severity of SMA is in direct correlation with the expression level of the SMN protein, either from the SMN1 gene or a different spliced form of SMN from the SMN2 gene. The SMN complex interacts with various protein substrates such as Sm and Lsm proteins of the spliceosomal snRNPs, fibrillarin, GAR1, RNA helicase A, the human hnRNP proteins (hnRNPQ, U and R), coilin and p53.

Disclaimer

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.

General description

Mouse monoclonal clone 2B1 anti-SMN antibody recognizes human, mouse, and Xenopus survival of motor neurons proteins.

Immunogen

recombinant human SMN

Physical form

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15mM sodium azide.

Specificity

The antibody recognizes human, mouse, and Xenopus SMN.

Storage and Stability

For continuous use, store at 2-8 °C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing, or storage in "frostfree" freezers, is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilution samples should be discarded if not used within 12 hours.