Product Description

SMN1 Polyclonal Antibody

Sizes: 50µl, 100µl

Catalogue Numbers: BS76703-50, BS76703-100

Product: 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Swiss-Prot: Q16637

Host: Rabbit

Reactivity: Human, Mouse, Rat

Applications: WB, IHC

All Applications: WB,1:500 - 1:2000 | IHC,1:50 - 1:200

Background: This gene is part of a 500 kb inverted duplication on chromosome 5q13. 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. 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.

Purification and Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity: Unmodification

Bioworld Molecular Weight: 32kDa

Note: For research use only, not for use in diagnostic procedure.

Extra Notes: Western blot analysis of extracts of various cell lines, using SMN1 antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG at 1:10000 dilution.
Lysates/proteins: 25ug per Lane.
Blocking buffer: 3% non-fat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 90s., Immunohistochemistry of paraffin-embedded rat lung using SMN1 antibody at dilution of 1:100. Perform microwave antigen retrieval with 10 mM PBS buffer pH 7.2 before commencing with IHC staining protocol., Immunohistochemistry of paraffin-embedded human breast using SMN1 antibody at dilution of 1:100. Perform microwave antigen retrieval with 10 mM PBS buffer pH 7.2 before commencing with IHC staining protocol., Immunohistochemistry of paraffin-embedded mouse kidney using SMN1 antibody at dilution of 1:100. Perform microwave antigen retrieval with 10 mM PBS buffer pH 7.2 before commencing with IHC staining protocol.

Alternative Name: SMN1; BCD541; GEMIN1; SMA; SMA1; SMA2; SMA3; SMA4

Immunogen: Recombinant fusion protein of human SMN1(NP_000335.1).

Conjugate: Unconjugated

Modification: Unmodified