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You are here:Home » Antibodies » Abs to RNA Processing » Anti -SMN1 (Survival of Motor Neuron Protein, Component of Gems 1, Gemin-1, SMNT) (Azide Free)

Anti -SMN1 (Survival of Motor Neuron Protein, Component of Gems 1, Gemin-1, SMNT) (Azide
Free)

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Specifications

Clone Host Grade Applications
Monoclonal Mouse Affinity Purified E B IH IF
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]
Catalog #S8495-02A
ApplicationsSuitable for use in ELISA, Immunofluorescence, Immunohistochemistry (paraffin), and Western Blot. Other applications not tested.
Recommended DilutionImmunohistochemistry (paraffin): 1.5ug/ml
ELISA: 1ug/ml-3ng/ml
Western Blot: 1:500-1:1,000
Optimal dilutions to be determined by the researcher.
Storage and StabilityMay be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Clone TypeMonoclonal
IsotypeIgG2a
Clone No11C825
HostMouse
SourceHuman
ConcentrationAs reported
FormSupplied as a liquid in PBS, pH 7.2. No preservative added (Azide free).
PurityPurified by immunoaffinity chromatography.
ImmunogenRecombinant protein corresponding to a portion of amino acids within aa1-283 of human SMN1.
SpecificityRecognizes human SMN1.
Important NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.


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