Wiskott-Aldrich Syndrome (Eczema-thrombocytopenia) (WAS) Antikörper

Antigen: Wiskott-Aldrich Syndrome (Eczema-thrombocytopenia) (WAS)

Synonyme: THC, IMD2, THC1, WASP

Klonalität: Polyklonal

Wirt: Ziege

Reaktivität: Human, Maus, Ratte (Rattus)

Applikation: Enzyme Immunoassay (EIA), Western Blot (WB)

Produktnummer: ABIN268835

Produktbeschreibung

Weitere Bezeichnung: WAS / IMD2

Gen-ID: 7454, 117538

Swiss-Prot: NP_0003681

Immunogen: Synthetic peptide C-SPADKKRSGKKKI, representing the Internal of the human proteinsequence according to NP_000368.1. Reference Sequence: NP_000368.1.

Isotyp: IgG  (Passende Sekundärantikörper)

Beschreibung: The Wiskott-Aldrich syndrome (WAS) family of proteins share similar domain structure,and are involved in transduction of signals from receptors on the cell surface to the actincytoskeleton. The presence of a number of different motifs suggests that they areregulated by a number of different stimuli, and interact with multiple proteins. Recentstudies have demonstrated that these proteins, directly or indirectly, associate with thesmall GTPase, Cdc42, known to regulate formation of actin filaments, and thecytoskeletal organizing complex, Arp2/3. Wiskott-Aldrich syndrome is a rare, inherited,X-linked, recessive disease characterized by immune dysregulation andmicrothrombocytopenia, and is caused by mutations in the WAS gene. The WAS geneproduct is a cytoplasmic protein, expressed exclusively in hematopoietic cells, whichshow signalling and cytoskeletal abnormalities in WAS patients. A transcript variantarising as a result of alternative promoter usage, and containing a different 5' UTRsequence, has been described, however, its full-length nature is not known. Alternate Names: anti-WAS antibody, anti-THC antibody, anti-IMD2 antibody, anti-WASP antibody,anti-Wiskott-Aldrich syndrome (eczema-thrombocytopenia) antibody,anti-thrombocytopenia 1 (X-linked) antibody, anti-Wiskott-Aldrich syndrome(eczema-thrombocytopenia) protein antibody.

Spezifität: Species Reactivity: Expected from sequence similarity: Human, Mouse, Rat and Dog.

Anwendungen

Applikationshinweise: Suggested working dilutions: ELISA 1:128,000,Western Blot 0.03-0.1ug/ml

Konzentration: 0.5 mg/ml

Buffer: Tris-saline containing 0.02% Na azide [pH7.3] with 0.5% BSA

Lagerung: Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.

Beschränkungen: Nur für Forschungszwecke einsetzbar