SMN Antibody

Product Discontinued

SMN Antibody Summary

• Immunogen: Peptide with sequence C-DESENSRSPGNKSDN corresponding to internal region according to NP_075012.1, NP_000335.1, NP_075013.1, NP_075014.1, NP_075015.1, NP_059107.1.
• Specificity: This antibody is expected to recognise isoforms b and d of SMN1 (NP_075012.1 and NP_000335.1) and all reported isoforms of SMN2 (NP_075013.1, NP_075014.1, NP_075015.1 and NP_059107.1).
• Isotype: IgG
• Clonality: Polyclonal
• Host: Goat
• Gene: SMN1
• Purity: Immunogen affinity purified

Applications/Dilutions

• Dilutions:
  • Peptide ELISA Detection limit 1:32000
  • Western Blot 0.3 - 1 ug/ml
• Application Notes: WB: Approx. 26 kDa band observed in human brain (cerebellum) lysates (calculated MW of 28.5 kDa band according to NP_075012.1 and NP_075014.1).
• Control:

  SMN Overexpression Lysate
  Human Brain Cerebellum Whole Tissue Lysate (Adult Whole Normal)

Packaging, Storage & Formulations

• Storage: Store at -20C. Avoid freeze-thaw cycles.
• Buffer: Tris (pH 7.3), 0.5% BSA
• Preservative: 0.02% Sodium Azide
• Concentration: 0.5 mg/ml
• Purity: Immunogen affinity purified

Alternate Names for SMN Antibody

• Gemin-1
• Kugelberg-Welander disease)
• SMNC
• survival motor neuron protein
• survival of motor neuron 1, telomeric

Background

SMN1/SMN2 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. While mutations in the telomeric copy are associated with spinal muscular atrophy, mutations in this gene, the centromeric copy, do not lead to disease. This gene 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. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The full length 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.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Publications for SMN Antibody (NB100-57859)(2)

Publications using NB100-57859

• Kobayashi DT, Olson RJ, Sly L et al. Utility of survival motor neuron ELISA for spinal muscular atrophy clinical and preclinical analyses. PLoS ONE. 2011-01-01 [PMID: 21904622]
• Setola V, Terao M, Locatelli D et al. Axonal-SMN (a-SMN), a protein isoform of the survival motor neuron gene, is specifically involved in axonogenesis. Proc Natl Acad Sci U S A 2007-02-06 [PMID: 17261814]

FAQs for SMN Antibody (NB100-57859). (Showing 1 - 1 of 1 FAQs).

1. Could you recommond the Human specific SMN antibody? It will only recognize human SMN, without cross reaction with mouse smn.
   • We do not have an SMN antibody that has been validated NOT to work in mouse samples. When an antibody is found to not react with a certain species, we mark the data sheet with a (-) next to the species' name. Unfortunately, the SMN antibodies that do not list mouse have not been tested in mouse, and may react.

Bioinformatics

• Gene Symbol: SMN1
• Entrez:
  • 6606(Human)
• Uniprot:
  • Q16637(Human)