SMN1 Antikörper

Produktnummer ABIN6568829

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch SMN1 in WB und IHC. Er zeigt eine Reaktivität gegenüber Human, Maus und Ratte.

Kurzübersicht für SMN1 Antikörper (ABIN6568829)

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

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser SMN1 Antikörper ist unkonjugiert

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

Produktdetails anti-SMN1 Antikörper

Aufreinigung: Affinity purification

Immunogen: Recombinant protein of human SMN2

Isotyp: IgG

Anwendungsinformationen

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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Konzentration: 1 mg/mL

Buffer: 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

Hintergrund:

Synonyms: BCD541,Component of gems 1,Gemin 1,Gemin-1,OTTHUMP00000125198,OTTHUMP00000223567,OTTHUMP00000223568,OTTHUMP00000224066,OTTHUMP00000226924,SMA 1,SMA 2,SMA 3,SMA 4,SMA,SMA@,SMA1,SMA2,SMA3,SMA4,SMN,SMN,SMN1,SMN2,SMNT,Survival motor neuron protein,Survival of motor neuron 1,telomeric,T-BCD541

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. While mutations in the telomeric copy are associated with spinal muscular atrophy, mutations in this gene, the centromeric copy, do not lead to disease. This gene 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 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.

Molekulargewicht:

Observed_MW: 35kDa

Calculated_MW: 27kDa/28kDa/30kDa/31kDa

Gen-ID: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization