SMN1 antibody (N-Term) (APC)

Catalog No. ABIN8043950

This Mouse Monoclonal antibody specifically detects SMN1 in FACS. It exhibits reactivity toward Human, Mouse and Rat.

Quick Overview for SMN1 antibody (N-Term) (APC) (ABIN8043950)

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

Reactivity: Human, Mouse, Rat

Host: Mouse

Clonality: Monoclonal

Conjugate: This SMN1 antibody is conjugated to APC

Application: Flow Cytometry (FACS)

Clone: 2B10

Product Details anti-SMN1 Antibody (APC)

Binding Specificity: N-Term

Purpose: Anti-SMN1/2 Antibody (monoclonal, 2B10) APC Conjugated

Specificity: No cross reactivity with other proteins.

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

Predicted Reactivity: identical to the related mouse,rat sequences.

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: IgG1

Application Details

Application Notes: Flow Cytometry, Optimal dilutions should be determined by end users.

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: Each vial contains 50 % glycerol, 0.9 % NaCl, 0.2 % Na2HPO4, 0.02 % 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.

Storage: -20 °C

Storage Comment: At -20°C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Expiry Date: 12 months

Target Details for SMN1

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

Alternative Name: SMN1

Background:

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.

Gene Full Name: survival of motor neuron 1, telomeric

Gene ID: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization