Anti-SMN1/2 Antibody | Boster Immunoleader PB9398 product information

product summary

request information :

company name :

Boster Immunoleader

product type :

antibody

product name :

Anti-SMN1/2 Antibody

catalog :

PB9398

quantity :

100 ug/vial

price :

240 USD

clonality :

polyclonal

host :

rabbit

reactivity :

human, mouse, rat

application :

western blot, immunohistochemistry - paraffin section

more info or order :

Boster Immunoleader product webpage

product information

Product Name :

Anti-SMN1/2 Antibody

Catalog Number :

PB9398

Clonality :

Polyclonal

Description short :

Rabbit IgG polyclonal antibody for Survival motor neuron protein(SMN1/2) detection. Tested with WB, IHC-P in Human;Mouse;Rat.

Price :

240 USD

Ig type :

Rabbit IgG

Size :

100 ug/vial

Form :

Lyophilized

Specificity :

No cross reactivity with other proteins.

Reactivity :

Reacts with: human, mouse, rat

Applications :

WB,IHC-P

Western blot :

Concentration: 0.1-0.5μg/ml; Tested Species: Human, Mouse, Rat

Immunohistochemistry(Paraffin-embedded S ... :

Concentration: 0.5-1μg/ml; Tested Species: Human, Mouse, Rat

Notes :

WB: The detection limit for SMN1/2 is approximately 0.1ng/lane under reducing conditions. Tested Species: In-house tested species with positive results. By Heat: Boiling the paraffin sections in 10mM citrate buffer, pH6.0, for 20mins is required for the staining of formalin/paraffin sections. Other applications have not been tested. Optimal dilutions should be determined by end users.

Immunogen :

A synthetic peptide corresponding to a sequence at the N-terminus of human SMN1/2(22-52aa RRGTGQSDDSDIWDDTALIKAYDKAVASFKH), identical to the related mouse and rat sequences.

Contents :

Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.

Purification :

Immunogen affinity purified.

Reconstitution :

Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Storage :

At -20˚C for one year. After reconstitution, at 4˚C for one month. It can also be aliquotted and stored frozen at -20˚C for a longer time. Avoid repeated freezing and thawing.

Relevant detection systems :

Boster provides a series of assays reacted with primary antibodies. Antibody can be supported by chemiluminescence kit EK1002 in WB, supported by SA1022 in IHC(P).

Background :

This gene is part of a 500 kb inverted duplication on chromosome 5q13. This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. However, mutations in this gene, the telomeric copy, are associated with spinal muscular atrophy; mutations in the centromeric copy do not lead to disease. The centromeric copy may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7, which is thought to be an exon splice enhancer. Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, 2a, 2b, and 3-8. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Multiple transcript variants encoding distinct isoforms have been described.

Reference :

1. Blauw, H. M., Barnes, C. P., van Vught, P. W. J., van Rheenen, W., Verheul, M., Cuppen, E., Veldink, J. H., van den Berg, L. H. SMN1 gene duplications are associated with sporadic ALS. Neurology 78: 776-780, 2012. 2. Boda, B., Mas, C., Giudicelli, C., Nepote, V., Guimiot, F., Levacher, B., Zvara, A., Santha, M., LeGall, I., Simonneau, M. Survival motor neuron SMN1 and SMN2 gene promoters: identical sequences and differential expression in neurons and non-neuronal cells. Europ. J. Hum. Genet. 12: 729-737, 2004.

Gene Name :

SMN1/2

Protein Name :

Survival motor neuron protein

Gene Full Name :

survival of motor neuron 1, telomeric/survival of motor neuron 2, centromeric

Synonyms :

Component of gems 1 antibody Component of gems 2 antibody Gemin 1 antibody Gemin-1 antibody SMA antibody SMA1 antibody SMA3 antibody SMN antibody SMN_HUMAN antibody SMN1 antibody SMN2 antibody SMNC antibody SMNT antibody Survival motor neuron protein antibody survival of motor neuron 1, telomeric antibody survival of motor neuron 2, centromeric antibody

Uniprot ID :

Q16637

more info or order :

Boster Immunoleader product webpage