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Home Antibody All anti-SMN1 antibodies

Anti-SMN1 Antibody

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Specifications Citations Related Products Product Documents
SKU Description Amount Price Availability*  
TA303208
  • Goat Polyclonal Antibody against SMN1 / SMN2
  • Free Sample of Positive Control: HEK293T cell transient overexpression lysate (LC424781) , 20ug
100ug 325 3-7 Days
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WB(1)
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Also for SMN1 (NM_000344)
cDNA Clone shRNA/siRNA Lysate Protein Antibody

OriGene Data

Immunogen Peptide with sequence C-DESENSRSPGNKSDN, from the internal region of the protein sequence according to NP_075012.1; NP_000335.1; NP_075013.1; NP_075014.1; NP_075015.1; NP_059107.1.
Clone Name IsotypeGoat IgG
Species ReactivityHuman Concentration0.5 mg/ml
Guaranteed Application *WB Suggested DilutionsELISA: 1:1,000. WB: 2-4µg/ml.
BufferSupplied at 0.5 mg/ml in Tris saline, 0.02% sodium azide, pH7.3 with 0.5% bovine serum albumin.
Purification Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide. Supplied at 0.5 mg/ml in Tris saline, 0.02% sodium azide, pH7.3 with 0.5% bovine serum albumin. Aliquot and store at -20°C. Minimize freezing and thawing.

Reference Data

Target NameHomo sapiens survival of motor neuron 1, telomeric (SMN1), transcript variant d
Alternative NameBCD541; GEMIN1; SMA; SMA1; SMA2; SMA3; SMA4; SMA@; SMN; SMNT; T-BCD541; TDRD16A
Database LinkNP_000335
FunctionThis 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 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 dorearrangements 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 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. [provided by RefSeq].
Related Pathway

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WB Image
TA303208 (2µg/ml) staining of Human Brain (Cerebellum) lysate (35µg protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.

 

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