SMN2 Antikörper (AA 1-197)

Produktnummer ABIN6148135

Produktdetails anti-SMN2 Antikörper

Target: SMN2 (Survival of Motor Neuron 2, Centromeric (SMN2))

Bindungsspezifität: AA 1-197

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser SMN2 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

Sequenz: MAMSSGGSGG GVPEQEDSVL FRRGTGQSDD SDIWDDTALI KAYDKAVASF KHALKNGDIC ETSGKPKTTP KRKPAKKNKS QKKNTAASLQ QWKVGDKCSA IWSEDGCIYP ATIASIDFKR ETCVVVYTGY GNREEQNLSD LLSPICEVAN NIEQNAQENE NESQVSTDES ENSRSPGNKS DNIKPKSAPW NSFLPPP

Kreuzreaktivität: Human, Maus, Ratte

Produktmerkmale: Polyclonal Antibodies

Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 1-197 of human SMN2 (NP_059107.1).

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB,1:500 - 1:2000,IHC,1:50 - 1:200,IF,1:50 - 1:200

Kommentare:

HIGH QUALITY

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: PBS with 0.02 % sodium azide,50 % glycerol, pH 7.3.

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Avoid freeze / thaw cycles.

Details zu SMN2

Target: SMN2 (Survival of Motor Neuron 2, Centromeric (SMN2))

Andere Bezeichnung: SMN2 (SMN2 Produkte)

Synonyme: BCD541 antikoerper, C-BCD541 antikoerper, GEMIN1 antikoerper, SMNC antikoerper, TDRD16B antikoerper, bcd541 antikoerper, c-bcd541 antikoerper, gemin-1 antikoerper, smn2 antikoerper, smnc antikoerper, survival of motor neuron 2, centromeric antikoerper, survival of motor neuron 2, centromeric L homeolog antikoerper, SMN2 antikoerper, smn2.L 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 telomeric and centromeric copies of this gene are nearly identical and encode the same protein. While mutations in the telomeric copy are associated with spinal muscular atrophy, mutations in this gene, the centromeric copy, do not lead to disease. 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 full length 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. Four transcript variants encoding distinct isoforms have been described.,SMN2,BCD541,C-BCD541,GEMIN1,SMNC,TDRD16B,SMN2

Molekulargewicht: 27 kDa/28 kDa/30 kDa/31 kDa

Gen-ID: 6607

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization