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- Antibody type:Primary
- Antigen name:survival of motor neuron 1, telomeric
- Antigen symbol:Survival of Motor Neuron 1
- Clonality:Polyclonal
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Western blot:Yes
- Form:Lyophilized
- Gene ID:6606
- Antigen synonyms:SMA@|SMA|SMA3|T-BCD541|SMN|GEMIN1|SMA1|SMA2|SMA4|TDRD16A|SMNT|BCD541
- UniProtKB:Q16637
- Storage temperature:At -20˚C for one year. After reconstitution, at 4˚C for one month. It can also be aliquotted and stored frozen at -20˚C for a longer time. Avoid repeated freezing and thawing.
- Immunogen:A synthetic peptide corresponding to a sequence at the N-terminus of human SMN1/2(22-52aa RRGTGQSDDSDIWDDTALIKAYDKAVASFKH), identical to the related mouse and rat sequences.
- Purification:Immunogen affinity purified.
- Size:100 µg/vial
- Pk:100 µG
Rabbit IgG polyclonal antibody for Survival motor neuron protein(SMN1/2) detection. Tested with WB, IHC-P in Human;Mouse;Rat.
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. Multiple transcript variants encoding distinct isoforms have been described.
Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
WB: The detection limit for SMN1/2 is approximately 0.1ng/lane under reducing conditions.
Tested Species: In-house tested species with positive results.
By Heat: Boiling the paraffin sections in 10mM citrate buffer, pH6.0, for 20mins is required for the staining of formalin/paraffin sections.
Other applications have not been tested.
Optimal dilutions should be determined by end users.
Type: Primary
Antigen: SNAI1
Clonality: Polyclonal
Clone:
Conjugation:
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat