Anti-SMN1/2 Antibody Picoband® (monoclonal, 2B10)

SKU: M03420-1

Size: 100 μg

Storage: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: IHC,ICC,WB

Application Details: Western blot, 0.1-0.5μg/ml
Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml
Immunocytochemistry, 0.5-1μg/ml

Gene Name: SMN1

Specificity: No cross reactivity with other proteins.

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.

Immunogen: A synthetic peptide corresponding to a sequence at the N-terminus of human SMN1/2, identical to the related mouse and rat sequences.

Clonality: Monoclonal

Contents: Each vial contains 4mg Trehalose, 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.

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.

Uniprot ID: Q16637

Host: Mouse

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate: This is the unconjugated antibody. It is also available with 10 conjugation options, including Biotin, Cy3, Fluoro488, Fluoro550, Fluoro594, FITC, HRP, APC, PE, and Fluoro647. A carrier-free (BSA-free) version is also available. Additional charges may apply for some options. Please contact us for a quote

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Mouse IgG1

Phospho_site:

Clone Number: Clone: 2B10

Observed Molecular Weight: 39 kDa

Calculated Molecular Weight: 32 kDa

Gene ID: 6606

Protein Name: Survival motor neuron protein

Gene Full Name: survival of motor neuron 1, telomeric

Synonyms: C BCD541, Component of gems 1, Gemin 1, SMN, SMN1, SMN1,SMN, SMN2, SMNC, SMNT, Survival motor neuron protein