SMN1 antibody

Catalog No. ABIN7270651

This Rabbit Monoclonal antibody specifically detects SMN1 in WB and IHC. It exhibits reactivity toward Human.

Quick Overview for SMN1 antibody (ABIN7270651)

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

Reactivity: Human

Host: Rabbit

Clonality: Monoclonal

Conjugate: This SMN1 antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (IHC)

Product Details anti-SMN1 Antibody

Purpose: SMN1 Rabbit mAb

Cross-Reactivity: Human, Rat

Characteristics: Monoclonal Antibodies

Purification: Affinity purification

Immunogen: A synthesized peptide derived from human SMN1

Isotype: IgG

Application Details

Application Notes: WB,1:500 - 1:2000,IHC,1:50 - 1:200

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: PBS with 0.02 % sodium azide,0.05 % BSA,50 % glycerol, pH 7.3.

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: Store at -20°C. Avoid freeze / thaw cycles.

Target Details for SMN1

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

Alternative Name: SMN1

Background: This gene is part of a 500 kb inverted duplication on chromosome 5q13. 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. 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.,BCD541, GEMIN1, SMA, SMA1, SMA2, SMA3, SMA4, SMA@, SMN, SMNT, T-BCD541, TDRD16A,SMN1

Molecular Weight: 36kDa

Gene ID: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization