anti- SMN antibody

Size

2X100μg

Catalog no#

FNab08031

Price

320 EUR

Uniprot ID

Q16637

Calculated MW

38 kDa

Form

liquid

French translation

anticorps

Specificity

Human, Mouse

Tested Application

ELISA, WB, IHC

Purification

Immunogen affinity purified

Purity

≥95% as determined by SDS-PAGE

Recommended dilution

WB: 1:500 - 1:2000; IHC: 1:50 - 1:200

Immunogen

survival of motor neuron 2, centromeric

Image1

Immunohistochemical of paraffin-embedded human kidney using FNab08031(SMN2 antibody) at dilution of 1:100

Storage

PBS with 0.02% sodium azide and 50% glycerol pH 7.3 , -20℃ for 24 months (Avoid repeated freeze / thaw cycles.)

Image4

K-562 cells were subjected to SDS PAGE followed by western blot with FNab08031(SMN2 antibody) at dilution of 1:1000

Synonyms

C BCD541, Component of gems 1, FLJ76644, Gemin 1, SMN, SMN1, SMN1, SMN, SMN2, SMNC, SMNT, Survival motor neuron protein

Properties

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

Description

This antibody needs to be stored at + 4°C in a fridge short term in a concentrated dilution. Freeze thaw will destroy a percentage in every cycle and should be avoided.Antibody for research use.

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. 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.