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

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

Synonyme: THC, IMD2, THC1, WASP

Klonalität: Polyklonal

Wirt: Kaninchen

Reaktivität: Human

Applikation: Western Blot (WB)

Produktnummer: ABIN250688

Produktbeschreibung

Weitere Bezeichnung: WAS / IMD2

Gen-ID: 7454

Swiss-Prot: NP_0003681

Immunogen: The epitope recognized by this antibody isphosphorylated Serine 483, phosphorylatedSerine 484 and surrounding residues of human Wiskott-Aldrich syndrome using thenumbering given in entry NP_000368.1 (GeneID 7454).Phosphorylated:Ser484.

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',UTR sequence, has been described, however, its full-length nature is not known. Alternate Names: anti-WAS antibody, anti-WASP antibody, anti-Wiskott-Aldrich Syndrome(eczema-thrombocytopenia) Protein antibody, anti-THC antibody,anti-Thrombocytopenia 1 (X-linked) antibody, anti-IMD2 antibody.

Spezifität: Species Reactivity: Reacts with Human. Reactivity with Mouse WASP phosphorylated at the residuescorresponding to S483 and S484 of Human WASP is likely owing to conservation ofsequence between Human and Mouse WASP in the vicinity of these residues.

Anwendungen

Applikationshinweise: Suggested working dilutions: Western Blot 1:1,000 - 1:10,000

Konzentration: 1 mg/ml

Buffer: Tris-citrate/phosphate buffer, pH 7 to 8. Preservative: 0.09% Sodium Azide.

Lagerung: Store at 4C. Do not freeze.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Bilder

Detection of Human Phospho-WASP-S483/S484 by Western Blot. Samples: Whole cell lysate (50 mcg/lane) from U937 and Jurkat cells. Lysates were either untreated, mock treated with phosphatase or treated with phosphatase. Antibody: Affinity purified rabbit anti-phospho WASP-S483/S484 ABIN250688 used at 1 mcg/ml. Detection: Chemiluminescence with an exposure time of 30 seconds.