SMN1 Antibody from ANTIBODIES-ONLINE GmbH

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Antigenic SpecificitySMN1
Clone7B10
Host SpeciesMouse
Reactive Specieshuman
IsotypeIgG1
Formatunconjugated
Size200 µg
Concentrationlot specific
ApplicationsImmunoprecipitation, Western Blotting
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DescriptionThe antibody was used extensively to purify and characterize the macromolecular SMN complex from cultured human cells. The antibody binds with high affinity and specificity to its epitope, which comprises a sequence of up to 9 amino acids in the N-terminal part of human SMN. It allows the native elution of bound antigen with an excess of the cognate peptide. Via addition of the 7B10 epitope (TagIt epitope) to other proteins this purification strategy can also be exploited for purification of these fusion proteins and RNA-protein complexes from cells lacking this epitope on endogenous proteins, such as cells derived from C. elegans and Drosophila. Target Information: 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. 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. Two transcript variants encoding distinct isoforms have been described. [provided by RefSeq, Sep 2008].
ImmunogenRecombinant human full-length survival motor neuron protein (SMN).
Other NamesSMN2|survival of motor neuron 2|centromeric|survival motor neuron protein|LOC461829|Survival of Motor Neuron 1, Telomeric|SMN1|Smn|survival motor neuron 1|BCD541|GEMIN1|SMA|SMA1|SMA2|SMA3|SMA4|SMA@|SMNT|T-BCD541|TDRD16A|component of gems 1|gemin-1|survival motor neuron 1 protein|tudor domain containing 16A|AI849087|survival of motor neuron protein|survival of motor neuron 2, centromeric|survival motor neuron protein-like|LOC100348318|survival motor neuron|LOC100713418|survival of motor neuron 1|telomeric|LOC100065744|LOC102176643|QtsA-10002|fa12d01|wu:fa12d01|survival motor neuron protein 1
Gene, Accession #Gene ID: 6606
Catalog #ABIN6253498
Price$431
Order / More InfoSMN1 Antibody from ANTIBODIES-ONLINE GmbH
Product Specific Referencesn/a
ANTIBODIES-ONLINE GmbH
ANTIBODIES-ONLINE GmbH
ANTIBODIES-ONLINE GmbH
Schloss-Rahe-Str. 15
52072 Aachen GERMANY
P: +49 (0)241 95 163 153
F: +49 (0)241 95 163 155

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