Products

SMN antibody

Synonyms:C BCD541 antibody, Component of gems 1 antibody, FLJ76644 antibody, Gemin 1 antibody, SMN antibody, SMN1 antibody, SMN1 antibody, SMN antibody, SMN2 antibody, SMNC antibody, SMNT antibody, Survival motor neuron protein antibody
Catalogue No.:FNab08031Reativity:Human, Mouse
Host:RabbitTested Application:ELISA, WB, IHC
Clonality:polyclonalIsotype:IgG
  • SPECIFICATIONS
Product Name
SMN antibody
Catalogue No.
FNab08031
Size
100μg
Form
liquid
Purification
Immunogen affinity purified
Purity
≥95% as determined by SDS-PAGE
Clonality
polyclonal
Isotype
IgG
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20℃ for 12 months (Avoid repeated freeze / thaw cycles.)
Immunogen
Immunogen
survival of motor neuron 2, centromeric
Alternative Names
C BCD541 antibody, Component of gems 1 antibody, FLJ76644 antibody, Gemin 1 antibody, SMN antibody, SMN1 antibody, SMN1 antibody, SMN antibody, SMN2 antibody, SMNC antibody, SMNT antibody, Survival motor neuron protein antibody
UniProt ID
Q16637
Observed MW
38 kDa
Application
Tested Applications
ELISA, WB, IHC
Recommended dilution
WB: 1:500 - 1:2000; IHC: 1:50 - 1:200
Validated Images
K-562 cells were subjected to SDS PAGE followed by western blot with FNab08031(SMN2 antibody) at dilution of 1:1000
Immunohistochemical of paraffin-embedded human kidney using FNab08031(SMN2 antibody) at dilution of 1:100
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.