SMN1 Antikörper

Produktnummer ABIN2854909

Produktdetails anti-SMN1 Antikörper

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser SMN1 Antikörper ist unkonjugiert

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

Kreuzreaktivität: Human

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

Aufreinigung: Purified by antigen-affinity chromatography.

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

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: 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.

Kommentare:

Positive Control: 293T , A431 , HeLa , HepG2

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 mg/mL

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

Konservierungsmittel: Thimerosal (Merthiolate)

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

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: 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.

Referenzen für anti-SMN1 Antikörper (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).

Details zu SMN1

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

Andere Bezeichnung: survival of motor neuron 1, telomeric (SMN1 Produkte)

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

Hintergrund: 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.

Molekulargewicht: 32 kDa

Gen-ID: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization