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Wiskott-Aldrich Syndrome Protein Mouse anti-Human, Unlabeled, Clone: 5A5, BD

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Description

Mouse Monoclonal Antibody

Wiskott-Aldrich syndrome (WAS) is an X-linked recessive immunodeficiency disease caused by mutations in the gene encoding WAS protein (WASP). The disease is characterized by a spectrum of clinical signs, including thrombocytopenia, eczema, susceptibility to opportunistic and pyogenic infections, and B-cell lymphomas associated with Epstein-Barr virus. Furthermore, patients' blood cells display morphological abnormalities that can be associated with an impaired cytoskeleton. WASP is a member of a family of highly conserved proteins that link signaling pathways to the actin cytoskeleton. These members include WASP, N-WASP (neuronal), and SCAR/WAVE isoforms (Suppressor of cAMP Receptor/WASP family Verprolin-homologous protein) that share two main regions of homology: a proline-rich domain and a carboxyl terminal domain that binds to the Arp2/3 complex. The Arp2/3 complex initiates actin filament assembly in motile cells and formation of the immunological synapse between activated T lymphocytes and antigen-presenting cells. WASP is a central regulator of the actin cytoskeleton in hematopoietic cells that is itself regulated by multiple signaling pathways.

The 5A5 antibody recognizes human WASP; it does not cross react with N-WASP. It has been reported to detect WASP in lysates of hematopoietic cells and cell lines, except for neutrophils, from normal donors, but not from a group of patients having mutations of the WAS gene.

TRUSTED_SUSTAINABILITY

Specifications

Antigen Wiskott-Aldrich Syndrome Protein
Applications Western Blot
Classification Monoclonal
Clone 5A5
Concentration 0.25mg/mL
Conjugate Unconjugated
Description WASP
Formulation Aqueous buffered solution containing BSA, glycerol, and ≤0.09% sodium azide.
Host Species Mouse
Immunogen Human WASP Recombinant Protein
Purification Method Affinity Purified
Quantity 0.1 mg
Regulatory Status RUO
Primary or Secondary Primary
Target Species Human
Content And Storage Store undiluted at 4°C
Isotype IgG2a κ
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