S2944
Anti-SMN antibody, Mouse monoclonal
clone 2B1, purified from hybridoma cell culture
Synonyme(s) :
Anti-Survival of Motor Neurons
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About This Item
Produits recommandés
Source biologique
mouse
Conjugué
unconjugated
Forme d'anticorps
purified from hybridoma cell culture
Type de produit anticorps
primary antibodies
Clone
2B1, monoclonal
Forme
buffered aqueous solution
Espèces réactives
Xenopus, human, mouse
Concentration
~2 mg/mL
Technique(s)
microarray: suitable
western blot: 2-4 μg/mL using A431 cell extract
Isotype
IgG1
Conditions d'expédition
dry ice
Température de stockage
−20°C
Modification post-traductionnelle de la cible
unmodified
Informations sur le gène
human ... SMN1(6606) , SMN2(6607)
mouse ... Smn1(20595)
Description générale
Mouse monoclonal clone 2B1 anti-SMN antibody recognizes human, mouse, and Xenopus survival of motor neurons proteins.
Spécificité
The antibody recognizes human, mouse, and Xenopus SMN.
Immunogène
recombinant human SMN
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.
Actions biochimiques/physiologiques
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.
Forme physique
Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15mM sodium azide.
Stockage et stabilité
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.
Clause de non-responsabilité
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.
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Code de la classe de stockage
12 - Non Combustible Liquids
Classe de danger pour l'eau (WGK)
WGK 2
Point d'éclair (°F)
Not applicable
Point d'éclair (°C)
Not applicable
Certificats d'analyse (COA)
Recherchez un Certificats d'analyse (COA) en saisissant le numéro de lot du produit. Les numéros de lot figurent sur l'étiquette du produit après les mots "Lot" ou "Batch".
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Retrouvez la documentation relative aux produits que vous avez récemment achetés dans la Bibliothèque de documents.
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
Q Liu et al.
The EMBO journal, 15(14), 3555-3565 (1996-07-15)
Spinal muscular atrophy (SMA) is a common, often fatal, autosomal recessive disease leading to progressive muscle wasting and paralysis as a result of degeneration of anterior horn cells of the spinal cord. A gene termed survival of motor neurons (SMN)
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
Sergey Paushkin et al.
Current opinion in cell biology, 14(3), 305-312 (2002-06-18)
Spinal muscular atrophy is a common, often lethal, neurodegenerative disease that results from low levels of, or loss-of-function mutations in, the SMN (survival of motor neurons) protein. SMN oligomerizes and forms a stable complex with five additional proteins: Gemins 2-6.
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