SMN1 Antikörper (AA 80-150)

Produktnummer ABIN7270652

Produktdetails anti-SMN1 Antikörper

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

Bindungsspezifität: AA 80-150

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser SMN1 Antikörper ist unkonjugiert

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

Verwendungszweck: SMN1 Rabbit pAb

Sequenz: SQKKNTAASL QQWKVGDKCS AIWSEDGCIY PATIASIDFK RETCVVVYTG YGNREEQNLS DLLSPICEVA N

Kreuzreaktivität: Human, Maus, Ratte

Produktmerkmale: Polyclonal Antibodies

Aufreinigung: Affinity purification

Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 80-150 of human SMN1 (NP_000335.1).

Isotyp: IgG

Anwendungsinformationen

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

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 SMN1

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

Andere Bezeichnung: SMN1 (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. 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. 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. Multiple transcript variants encoding distinct isoforms have been described.,SMN1,BCD541,GEMIN1,SMA,SMA1,SMA2,SMA3,SMA4,SMN1

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

Gen-ID: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization