FNab08030

antibody from FineTest Biotech

Targeting: SMN2 BCD541, GEMIN1, SMNC, TDRD16B

Western blot (Data presented on provider website)

ELISA (Data presented on provider website)

Immunohistochemistry (Data presented on provider website)

Antibody Data

Product number

FNab08030

Provider

FineTest Biotech

Product name

SMN antibody

Antibody type

Polyclonal

Antigen

Recombinant protein fragment

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.

Reactivity

Human, Mouse, Rat

Host

Rabbit

Conjugate

Unconjugated

Antigen sequence

survival of motor neuron 2, centrom
eric


Epitope

IgG

Isotype

IgG

Vial size

100¦Ìg

Concentration

Immunogen affinity purified

Storage

PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20¡æ for 12 months (Avoid repeated freeze / thaw cycles.)

Handling

PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20¡æ for 12 months (Avoid repeated freeze / thaw cycles.)