SMN1 antibody

Catalog No. ABIN2854909

Product Details anti-SMN1 Antibody

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

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This SMN1 antibody is un-conjugated

Application: Western Blotting (WB), Immunofluorescence (IF), Immunocytochemistry (ICC)

Cross-Reactivity: Human

Characteristics: Rabbit Polyclonal antibody to SMN1 (survival of motor neuron 1, telomeric)
SMN1 antibody

Purification: Purified by antigen-affinity chromatography.

Immunogen: Recombinant protein encompassing a sequence within the center region of human SMN1. The exact sequence is proprietary.

Isotype: IgG

Application Details

Application Notes: WB: 1:500-1:3000. ICC/IF: 1:100-1:1000. Optimal dilutions/concentrations should be determined by the researcher. Not tested in other applications.

Comment:

Positive Control: 293T , A431 , HeLa , HepG2

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 mg/mL

Buffer: 1XPBS ( pH 7), 40 % Glycerol, 0.01 % Thimerosal

Preservative: Thimerosal (Merthiolate)

Precaution of Use: This product contains Thimerosal (Merthiolate): a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4°C. For long-term storage, aliquot and store at -20°C or below. Avoid multiple freeze-thaw cycles.

References for anti-SMN1 antibody (ABIN2854909)

Zhang, Lin, Li, Lu, Guo, Dong, Zhao, He, Wang, Chen: "Application of urine cells in drug intervention for spinal muscular atrophy." in: Experimental and therapeutic medicine, Vol. 14, Issue 3, pp. 1993-1998, (2017) (PubMed).

Target Details for SMN1

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

Alternative Name: survival of motor neuron 1, telomeric (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: 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. Two transcript variants encoding distinct isoforms have been described.

Molecular Weight: 32 kDa

Gene ID: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization