SMN2 Polyclonal Antibody | Invitrogen PA5-119447 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

Invitrogen

other brands :

NeoMarkers, Lab Vision, Endogen, Pierce, BioSource International, Zymed Laboratories, Caltag, Molecular Probes, Research Genetics, Life Technologies, Applied Biosystems, GIBCO BRL, ABgene, Dynal, Affinity BioReagents, Nunc, Invitrogen, NatuTec, Oxoid, Richard-Allan Scientific, Arcturus, Perseptive Biosystems, Proxeon, eBioscience

product type :

antibody

product name :

SMN2 Polyclonal Antibody

catalog :

PA5-119447

quantity :

200 uL

price :

US 434.00

clonality :

polyclonal

host :

domestic rabbit

conjugate :

nonconjugated

reactivity :

human

application :

western blot, immunohistochemistry, immunohistochemistry - paraffin section

product information

Product Type :

Antibody

Product Name :

SMN2 Polyclonal Antibody

Catalog # :

PA5-119447

Quantity :

200 uL

Price :

US 434.00

Clonality :

Polyclonal

Purity :

Antigen affinity chromatography

Host :

Rabbit

Reactivity :

Human

Applications :

Immunohistochemistry (Paraffin): 5-20 ug/mL, Western Blot: 0.5-2 ug/mL

Species :

Human

Isotype :

IgG

Storage :

-20 C

Description :

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. 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. 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 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. Four transcript variants encoding distinct isoforms have been described.

Immunogen :

Recombinant protein Survival Of Motor Neuron 2, Centromeric. The antigen corresponds to amino acid range 16-251 of the target protein.

Format :

Liquid

Applications w/Dilutions :

Immunohistochemistry (Paraffin): 5-20 ug/mL, Western Blot: 0.5-2 ug/mL

Aliases :

BCD541; C-BCD541; Component of gems 1; GEMIN1; gemin-1; SMN; SMN1; SMN2; SMNC; SMNT; Survival motor neuron protein; Survival Of Motor Neuron 2; survival of motor neuron 2, centromeric; TDRD16B; tudor domain containing 16B; XLRS1