产品名称
Anti-SMN antibody, Mouse monoclonal, clone 2B1, purified from hybridoma cell culture
biological source
mouse
conjugate
unconjugated
antibody form
purified from hybridoma cell culture
antibody product type
primary antibodies
clone
2B1, monoclonal
form
buffered aqueous solution
species reactivity
Xenopus, human, mouse
concentration
~2 mg/mL
technique(s)
microarray: suitable
western blot: 2-4 μg/mL using A431 cell extract
isotype
IgG1
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... SMN1(6606), SMN2(6607)
mouse ... Smn1(20595)
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)
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.
The antibody recognizes human, mouse, and Xenopus SMN.
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.
Preparation Note
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.
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存储类别
12 - Non Combustible Liquids
wgk
WGK 2
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
常规特殊物品
此项目有
Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?
Burghes AHM and Beattie CE
Nature Reviews. Neuroscience, 10(8), 597-597 (2009)
Dione T Kobayashi et al.
PloS one, 6(8), e24269-e24269 (2011-09-10)
Genetic defects leading to the reduction of the survival motor neuron protein (SMN) are a causal factor for Spinal Muscular Atrophy (SMA). While there are a number of therapies under evaluation as potential treatments for SMA, there is a critical
Thomas O Crawford et al.
PloS one, 7(4), e33572-e33572 (2012-05-05)
The universal presence of a gene (SMN2) nearly identical to the mutated SMN1 gene responsible for Spinal Muscular Atrophy (SMA) has proved an enticing incentive to therapeutics development. Early disappointments from putative SMN-enhancing agent clinical trials have increased interest in
Matthew M Seavey et al.
Current protocols in pharmacology, Chapter 5, Unit 5-Unit 5 (2011-09-22)
Systemic Lupus Erythematosus (SLE) is a debilitating and often fatal autoimmune disease that involves multiple organ systems. It can develop for years before being diagnosed. Current treatments for SLE usually involve the use of cytotoxic or immunosuppressive agents that can
Amparo Garcia-Lopez et al.
Nature communications, 9(1), 2032-2032 (2018-05-26)
Modification of SMN2 exon 7 (E7) splicing is a validated therapeutic strategy against spinal muscular atrophy (SMA). However, a target-based approach to identify small-molecule E7 splicing modifiers has not been attempted, which could reveal novel therapies with improved mechanistic insight.
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