SMN1 / SMN2 (AA 22-52), (N-Term) antibody

Catalog No. ABIN5693315

Product Details

Target: SMN1 / SMN2

Binding Specificity: AA 22-52, N-Term

Reactivity: Human

Host: Mouse

Clonality: Monoclonal

Conjugate: Un-conjugated

Application: Western Blotting (WB)

Brand: Picoband™

Sequence: RRGTGQSDDS DIWDDTALIK AYDKAVASFK H

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

Characteristics: Mouse IgG monoclonal antibody for SMN1/2 detection. Tested with WB in Human.

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.

Clone: 2B10

Isotype: IgG1

Application Details

Application Notes:

Recommended Detection Systems: Enhanced Chemiluminescent Kit with anti-Rabbit IgG (ABIN921124) for Western blot.

Application Details: Western blot, 0.1-0.5 μg/mL

Restrictions: For Research Use only

Handling

Format: Lyophilized

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

Buffer: Each vial contains 4 mg Trehalose, 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.

Storage: 4 °C,-20 °C

Storage Comment: 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.

Target Details

Target: SMN1 / SMN2

Alternative Name: SMN1/2

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.

UniProt: Q16637