SMN1 antibody

Catalog No. ABIN2462049

Product Details anti-SMN1 Antibody

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

Reactivity: Human, Dog

Host: Rabbit

Clonality: Polyclonal

Conjugate: This SMN1 antibody is un-conjugated

Application: Western Blotting (WB), ELISA

Purification: Antibody is purified by peptide affinity chromatography method.

Immunogen: Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human SMN1.

Application Details

Application Notes: SMN1 antibody can be used for detection of SMN1 by ELISA at 1:62500. SMN1 antibody can be used for detection of SMN1 by western blot at 0.5 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.

Restrictions: For Research Use only

Handling

Format: Lyophilized

Reconstitution: Add 50 ?L of distilled water. Final antibody concentration is 1 mg/mL.

Concentration: 1 mg/mL

Buffer: Antibody is lyophilized in PBS buffer with 2 % sucrose.

Handling Advice: As with any antibody avoid repeat freeze-thaw cycles.

Storage: 4 °C/-20 °C

Storage Comment: For short periods of storage (days) store at 4 °C. For longer periods of storage, store SMN1 antibody at -20 °C.

Target Details for SMN1

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

Alternative Name: SMN1 (SMN1 Products)

Synonyms: SMN2 antibody, Smn antibody, SMN antibody, BCD541 antibody, GEMIN1 antibody, SMA antibody, SMA1 antibody, SMA2 antibody, SMA3 antibody, SMA4 antibody, SMA@ antibody, SMNT antibody, T-BCD541 antibody, TDRD16A antibody, AI849087 antibody, Gemin1 antibody, SMN1 antibody, QtsA-10002 antibody, fa12d01 antibody, smn antibody, wu:fa12d01 antibody, survival motor neuron protein antibody, survival of motor neuron 1, telomeric antibody, survival motor neuron 1 antibody, survival of motor neuron 2, centromeric antibody, survival motor neuron protein-like antibody, survival motor neuron antibody, LOC461829 antibody, Smn1 antibody, SMN1 antibody, SMN2 antibody, LOC100348318 antibody, SMN antibody, LOC100713418 antibody, LOC100065744 antibody, LOC102176643 antibody, smn1 antibody

Background: SMN1 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.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. 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. Two transcript variants are produced by this gene.

Molecular Weight: 28 kDa

Gene ID: 6606

NCBI Accession: NP_075012

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization