- HOST SPECIES: Rabbit
- SPECIES REACTIVITY: Dog, Human
- IMMUNOGEN: Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human SMN1.
- CONJUGATE: Unconjugated
- TESTED APPLICATIONS: ELISA, WB
- APPLICATION NOTE: SMN1 antibody can be used for detection of SMN1 by ELISA at 1:62500. SMN1 antibody can be used for detection of SMN1 by western blot at 0.5 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.
- POSITIVE CONTROL 1: Cat. No. 1211 - HepG2 Cell Lysate
- PREDICTED MOLECULAR WEIGHT: 28 kDa
- PURIFICATION: Antibody is purified by peptide affinity chromatography method.
- CLONALITY: Polyclonal
- PHYSICAL STATE: Liquid
- BUFFER: Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
- CONCENTRATION: batch dependent
- STORAGE CONDITIONS: For short periods of storage (days) store at 4°C. For longer periods of storage, store SMN1 antibody at -20°C. As with any antibody avoid repeat freeze-thaw cycles.
- NCBI OFFICIAL SYMBOL: SMN1
- ADDITIONAL NAMES: SMN1, BCD541, SMA, SMA1, SMA2, SMA3, SMA4, [email protected], SMN, SMNT, T-BCD541, GEMIN1, TDRD16A
- Protein Accession Number: NP_075012
- PROTEIN GI NUMBER: 13259512
- NCBI GENE ID NUMBER: 6606
- USER NOTE: Optimal dilutions for each application to be determined by the researcher.
- SMN1 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.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. 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 are produced by this gene.
- 1: Hirano, M., (2008) Arch. Neurol. 65 (3), 403-406.
- FOR RESEARCH USE ONLY
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- Disclaimer: This product is for research use only.
CATALOG NUMBER: 29-213
- Size: 100 ul
- List Price: $475.00
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