SMN1 Antikörper (N-Term) (APC)

Produktnummer ABIN8043950

Dieser Maus Monoklonal Antikörper detektiert spezifisch SMN1 in FACS. Es zeigt Reaktivität gegenüber Proben von Human, Maus und Ratte.

Kurzübersicht für SMN1 Antikörper (N-Term) (APC) (ABIN8043950)

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

Reaktivität: Human, Maus, Ratte

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser SMN1 Antikörper ist konjugiert mit APC

Applikation: Flow Cytometry (FACS)

Klon: 2B10

Produktdetails anti-SMN1 Antikörper (APC)

Bindungsspezifität: N-Term

Verwendungszweck: Anti-SMN1/2 Antibody (monoclonal, 2B10) APC Conjugated

Spezifität: No cross reactivity with other proteins.

Kreuzreaktivität (Details): No cross-reactivity with other proteins.

Homologie: identical to the related mouse,rat sequences.

Aufreinigung: Immunogen affinity purified.

Immunogen: A synthetic peptide corresponding to a sequence at the N-terminus of human SMN1/2, identical to the related mouse and rat sequences.

Isotyp: IgG1

Anwendungsinformationen

Applikationshinweise: Flow Cytometry, Optimal dilutions should be determined by end users.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: Each vial contains 50 % glycerol, 0.9 % NaCl, 0.2 % Na2HPO4, 0.02 % Sodium azide.

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: At -20°C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Haltbarkeit: 12 months

Details zu SMN1

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

Andere Bezeichnung: SMN1

Hintergrund:

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. 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. Multiple transcript variants encoding distinct isoforms have been described.

Gene Full Name: survival of motor neuron 1, telomeric

Gen-ID: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization