SMN1, SMN2 Antibody - N-terminal region (OABB01881)

Catalog No: OABB01881

Size:100UG

Anti-SMN1/2 Picoband antibody, OABB01881, Western blotting
All lanes: Anti SMN1/2(OABB01881) at 0.5ug/ml
Lane 1: Rat Brain Tissue Lysate at 50ug
Lane 2: Mouse Brain Tissue Lysate at 50ug
Lane 3: Rat Liver Tissue Lysate at 50ug
Lane 4: Mouse Liver Tissue Lysate at 50ug
Lane 5: 293T Whole Cell Lysate at 40ug
Lane 6: SMMC Whole Cell Lysate at 40ug
Lane 7: HEPG2 Whole Cell Lysate at 40ug
Lane 8: HELA Whole Cell Lysate at 40ug
Predicted bind size: 32KD
Observed bind size: 32KD

Anti-SMN1/2 Picoband antibody, OABB01881, IHC(P)
IHC(P): Human Mammary Cancer Tissue

Anti-SMN1/2 Picoband antibody, OABB01881, IHC(P)
IHC(P): Mouse Brain Tissue

Anti-SMN1/2 Picoband antibody, OABB01881, IHC(P)
IHC(P): Rat Brain Tissue

Product Info

• Tested Species Reactivity: Human, Mouse, Rat
• Predicted Species Reactivity: Hamster|Human|Mouse|Rat
• Product Format: Lyophilized. Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
• Clonality: Polyclonal
• Clone: Polyclonal
• Isotype: Rabbit IgG
• Host: Rabbit
• Application: Flow cytometry|Immunocytochemistry|Immunofluorescence|Immunohistochemistry|Western blot
• Additional Information: Notes: WB: The detection limit for SMN1/2 is approximately 0.1ng/lane under reducing conditions.
  Tested Species: In-house tested species with positive results.
  By Heat: Boiling the paraffin sections in 10mM citrate buffer, pH6.0, for 20mins is required for the staining of formalin/paraffin sections.
  Other applications have not been tested.
  Optimal dilutions should be determined by end users.
  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.
  
• Reconstitution and Storage: 2°C to 8°C|-20°C
• Immunogen: Polypeptide
• Purification: Affinity Purified
• Peptide Sequence: Synthetic peptide located within the following region: RRGTGQSDDSDIWDDTALIKAYDKAVASFKH
• Concentration: 500 ug/ml
• Specificity: No cross reactivity with other proteins.
• Application Info: Western blot: 0.1-0.5 ug/ml: Human, Mouse, Rat
  Immunohistochemistry (Paraffin-embedded Section): 0.5-1 ug/ml: Human, Mouse, Rat: By Heat
  Immunocytochemistry/Immunofluorescence: 2 ug/ml: Human
  Flow Cytometry: 1-3 ug/1x10^6 cells: Human
• 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.
  
• Storage Buffer: Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
• Description: Rabbit IgG polyclonal antibody for Survival motor neuron protein(SMN1/2) detection. Tested with WB, IHC-P, ICC/IF, FCM in Human;Mouse;Rat.

Target Info

• Gene Symbol: SMN1|SMN2
• Gene Full Name: survival of motor neuron 1, telomeric|survival of motor neuron 2, centromeric
• Alias Symbols: BCD541;C-BCD541;component of gems 1;gemin-1;GEMIN1;SMA;SMA@;SMA1;SMA2;SMA3;SMA4;SMN;SMNC;SMNT;survival motor neuron 1 protein;survival motor neuron protein;T-BCD541;TDRD16A;TDRD16B;tudor domain containing 16A;tudor domain containing 16B.
• NCBI Gene Id: 6606|6607
• Protein Name: Survival motor neuron protein
• Description of Target: 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. Also required for resolving RNA-DNA hybrids created by RNA polymerase II, that form R-loop in transcription terminal regions, an important step in proper transcription termination. May also play a role in the metabolism of small nucleolar ribonucleoprotein (snoRNPs).
• Uniprot ID: Q16637
• Molecular Weight: 31849 MW