SMN1 anticorps

N° du produit ABIN2854909

Détail du produit anti-SMN1 anticorps

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp SMN1 est non-conjugé

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

Réactivité croisée: Humain

Attributs du produit: Rabbit Polyclonal antibody to SMN1 (survival of motor neuron 1, telomeric)
SMN1 antibody

Purification: Purified by antigen-affinity chromatography.

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

Isotype: IgG

Information d'application

Indications d'application: 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.

Commentaires:

Positive Control: 293T , A431 , HeLa , HepG2

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 mg/mL

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

Agent conservateur: Thimerosal (Merthiolate)

Précaution d'utilisation: This product contains Thimerosal (Merthiolate): a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: 4 °C,-20 °C

Stockage commentaire: 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.

Références pour anticorps anti-SMN1 (ABIN2854909)

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

Détails sur SMN1

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

Autre désignation: survival of motor neuron 1, telomeric (SMN1 Produits)

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

Sujet: 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.

Poids moléculaire: 32 kDa

ID gène: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization