SMN1/2 Antibody / Survival of Motor Neuron 1/2

Basic informations

  • Size: 0.1mg
  • Catalog number: R32249
  • Price: 406.00EUR
SMN1/2 Antibody / Survival of Motor Neuron 1/2

Category

Antibody

Concentration

0.5mg/ml if reconstituted with 0.2ml sterile DI water

Form

Antigen affinity purified

Conjugation

Unconjugated

Clone

Polyclonal antibody

Recognised antigen

SMN1/2 / Survival of Motor Neuron 1/2

Host animal

Rabbit (Oryctolagus cuniculus)

Clonality

Polyclonal (rabbit origin)

Species reactivity

Human (Homo sapiens), Mouse (Mus musculus), Rat ; Due to limited knowledge and inability to test the antibody against all known species, we cannot guarantee that no other cross reactivity can occur.

Tested applications

WB, IHC-P

Recommended dilutions

Western blot: 0.1-0.5ug/ml,IHC (Paraffin): 0.5-1ug/ml

Notes

Optimal dilution of the SMN1/2 antibody should be determined by the researcher.

Intented use

This SMN1/2 antibodyis to be used only for research purposes and not for diagnostics..

Uniprot #

Q16637

Purity

Antigen affinity

Description

The Survival of Motor Neuron 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.

Immunogen

Amino acids RRGTGQSDDSDIWDDTALIKAYDKAVASFKH of human SMN1/2 were used as the immunogen for the SMN1/2 antibody.

Storage

After reconstitution, the SMN1/2 antibody may be kept for up to one month refrigerated at +4 degrees C.For long-term, aliquot and store at -20 deg. Celcius or lower. Cycles of freezing and thawing can denaturate the peptide chains of the antibodies and reduce their sensitivity and/or change their affinity. Prepare aliqotes in such a manner so that freeze-thaw cycles are minimized. Avoid repeated freezing and thawing.

Properties

If you buy Antibodies supplied by NJS poly they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.

French translation

anticorps