SMN1 antibody (N-Term)

Catalog No. ABIN3043933

This Rabbit Polyclonal antibody specifically detects SMN1 in WB, IHC, IF, ICC and FACS. It exhibits reactivity toward Human, Mouse and Rat.

Quick Overview for SMN1 antibody (N-Term) (ABIN3043933)

Target: SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This SMN1 antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (IHC), Immunofluorescence (IF), Immunocytochemistry (ICC), Flow Cytometry (FACS)

Product Details anti-SMN1 Antibody

Binding Specificity: AA 22-52, N-Term

Purpose: Anti-SMN1/2 Antibody Picoband®

Sequence: RRGTGQSDDS DIWDDTALIK AYDKAVASFK H

Cross-Reactivity (Details): No cross-reactivity with other proteins.

Characteristics: Anti-SMN1/2 Antibody Picoband® (ABIN3043933). Tested in Flow Cytometry, IF, IHC, ICC, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Purification: Immunogen affinity purified.

Immunogen: A synthetic peptide corresponding to a sequence at the N-terminus of human SMN1/2, identical to the related mouse and rat sequences.

Isotype: IgG

Application Details

Application Notes: Western blot, 0.1-0.5 μg/mL, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human, Mouse, Rat
Immunocytochemistry/Immunofluorescence, 2 μg/mL, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
1. Blauw, H. M., Barnes, C. P., van Vught, P. W. J., van Rheenen, W., Verheul, M., Cuppen, E., Veldink, J. H., van den Berg, L. H. SMN1 gene duplications are associated with sporadic ALS. Neurology 78: 776-780, 2012. 2. Boda, B., Mas, C., Giudicelli, C., Nepote, V., Guimiot, F., Levacher, B., Zvara, A., Santha, M., LeGall, I., Simonneau, M. Survival motor neuron SMN1 and SMN2 gene promoters: identical sequences and differential expression in neurons and non-neuronal cells. Europ. J. Hum. Genet. 12: 729-737, 2004.

Comment:

Antibody can be supported by chemiluminescence kit ABIN921124 in WB, supported by ABIN921231 in IHC(P).

Restrictions: For Research Use only

Handling

Format: Lyophilized

Reconstitution: Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Concentration: 500 μg/mL

Buffer: Each vial contains 5 mg BSA, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg Sodium azide.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handling Advice: Avoid repeated freezing and thawing.

Storage: 4 °C,-20 °C

Storage Comment: Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.

Target Details for SMN1

Target: SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

Alternative Name: SMN1

Background:

Synonyms: Survival motor neuron protein,Component of gems 1,Gemin-1,SMN1,SMN, SMNT,SMN2,SMNC,

Tissue Specificity: Expressed in a wide variety of tissues. Expressed at high levels in brain, kidney and liver, moderate levels in skeletal and cardiac muscle, and low levels in fibroblasts and lymphocytes. Also seen at high levels in spinal cord. Present in osteoclasts and mononuclear cells (at protein level). .

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. 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.

Sequence Similarities: Belongs to the SMN family.

Molecular Weight: 32 kDa

Gene ID: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization