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Goat Anti-SMN1 / SMN2 Antibody

Peptide-affinity purified goat antibody

  • WB - Goat Anti-SMN1 / SMN2 Antibody AF2008a
    AF2008a (1 µg/ml) staining of Human Cerebellum lysate (35 µg protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.
Product Information
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
Primary Accession Q16637
Other Accession NP_059107, 6606, 6607
Reactivity Human
Host Goat
Clonality Polyclonal
Concentration 0.5 mg/ml
Isotype IgG
Calculated MW 31849 Da
Additional Information
Gene ID 6606;6607
Other Names Survival motor neuron protein, Component of gems 1, Gemin-1, SMN1, SMN, SMNT
Format 0.5 mg IgG/ml in Tris saline (20mM Tris pH7.3, 150mM NaCl), 0.02% sodium azide, with 0.5% bovine serum albumin
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsGoat Anti-SMN1 / SMN2 Antibody is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name SMN1
Synonyms SMN, SMNT
Function The SMN complex plays a catalyst role in the assembly of small nuclear ribonucleoproteins (snRNPs), the building blocks of the spliceosome. Thereby, plays an important role in the splicing of cellular pre-mRNAs. Most spliceosomal snRNPs contain a common set of Sm proteins SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE, SNRPF and SNRPG that assemble in a heptameric protein ring on the Sm site of the small nuclear RNA to form the core snRNP. In the cytosol, the Sm proteins SNRPD1, SNRPD2, SNRPE, SNRPF and SNRPG are trapped in an inactive 6S pICln-Sm complex by the chaperone CLNS1A that controls the assembly of the core snRNP. Dissociation by the SMN complex of CLNS1A from the trapped Sm proteins and their transfer to an SMN-Sm complex triggers the assembly of core snRNPs and their transport to the nucleus. Ensures the correct splicing of U12 intron-containing genes that may be important for normal motor and proprioceptive neurons development. May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs).
Cellular Location Cytoplasm. Nucleus, gem. Nucleus, Cajal body. Cytoplasmic granule. Cytoplasm, myofibril, sarcomere, Z line. Note=Colocalizes with Actn at the Z-line of skeletal muscle (By similarity). Under stress conditions colocalizes with RPP20/POP7 in punctuated cytoplasmic granules Colocalized and redistributed with ZPR1 from the cytoplasm to nuclear gems (Gemini of coiled bodies) and Cajal bodies
Tissue Location Expressed in a wide variety of tissues. Expressed at high levels in brain, kidney and liver, moderate levels in skeletal and cardiac muscle, and low levels in fibroblasts and lymphocytes. Also seen at high levels in spinal cord. Present in osteoclasts and mononuclear cells (at protein level).
Research Areas
Citations (0)

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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. Two transcript variants encoding distinct isoforms have been described.


[Study of SMN gene in Chinese children with spinal muscular atrophy] Liu WL, et al. Zhongguo Dang Dai Er Ke Za Zhi, 2010 Jul. PMID 20637152. SMN, Gemin2 and Gemin3 associate with beta-actin mRNA in the cytoplasm of neuronal cells in vitro. Todd AG, et al. J Mol Biol, 2010 Sep 3. PMID 20620147. Splicing regulation of the survival motor neuron genes and implications for treatment of spinal muscular atrophy. Bebee TW, et al. Front Biosci, 2010 Jun 1. PMID 20515750. Molecular characterization of SMN copy number derived from carrier screening and from core families with SMA in a Chinese population. Sheng-Yuan Z, et al. Eur J Hum Genet, 2010 Sep. PMID 20442745. Large-scale population carrier screening for spinal muscular atrophy in Israel--effect of ethnicity on the false-negative rate. Sukenik-Halevy R, et al. Genet Test Mol Biomarkers, 2010 Jun. PMID 20373854.

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$ 325.00
Cat# AF2008a
(40 western blots)
Availability: 7-10 days
Bulk Size
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