Survival Motor Neuron Protein antibody
Mouse anti Human survival motor neuron protein antibody recognizes survival motor neuron 1 protein, also known as component of gems 1, gemin-1 and tudor domain containing 16A.
SMN1 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 SMN1 gene are nearly identical and encode the same protein. However, mutations in SMN1, 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 SMN1 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, Sep 2008).
Mouse anti Human survival motor neuron protein antibody detects a band of 39 kDa. The antibody has been extensively validated for western blotting using whole cell lysates.
SMN1 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 SMN1 gene are nearly identical and encode the same protein. However, mutations in SMN1, 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 SMN1 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, Sep 2008).
Mouse anti Human survival motor neuron protein antibody detects a band of 39 kDa. The antibody has been extensively validated for western blotting using whole cell lysates.
Product Details
- Target Species
- Human
- Western Blotting
- Anti survival motor neuron protein detects a band of approximately 39 kDa in MCF7 cell lysates
- Product Form
- Purified IgG - liquid
- Preparation
- Mouse monoclonal antibody prepared by affinity chromatography on Protein G from ascites
- Buffer Solution
- Phosphate buffered saline
- Preservative Stabilisers
- 0.09% Sodium Azide (NaN3)
- Immunogen
- Purified recombinant fragment of human SMN1 expressed in E. coli
Storage Information
- Storage
- Store undiluted at -20oC, avoiding repeated freeze thaw cycles.
- Guarantee
- 12 months from date of despatch
More Information
- UniProt
- Q16637
- Entrez Gene
- SMN2
- GO Terms
- GO:0005515 protein binding
- GO:0000245 spliceosome assembly
- GO:0000387 spliceosomal snRNP assembly
- GO:0003723 RNA binding
- GO:0005829 cytosol
- GO:0005681 spliceosomal complex
- GO:0008219 cell death
- GO:0015030 Cajal body
- GO:0034660 ncRNA metabolic process
- Acknowledgements
- PrecisionAb is a trademark of Bio-Rad Laboratories.
- Regulatory
- For research purposes only
Applications of Survival Motor Neuron Protein antibody
| Application Name | Verified | Min Dilution | Max Dilution |
|---|---|---|---|
| Western Blotting | 1/1000 |
The PrecisionAb label is reserved for antibodies that meet the defined performance criteria within Bio-Rad's ongoing antibody validation programme. Click here to learn how we validate our PrecisionAb range. Where this product has not been tested for use in a particular technique this does not necessarily exclude its use in such procedures. Further optimization may be required dependent on sample type.
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Secondary Antibodies Available
| Description | Product Code | Applications | Pack Size | List Price | Quantity |
|---|---|---|---|---|---|
| Goat anti Mouse IgG (H/L):HRP | STAR207P | WB | 2 ml |
