货号：557773

品牌：BD Pharmingen

规格：1EA

目录价：￥6052.00

市场价格：￥3812.76

会员价格：￥3328.60

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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.

Catalog Number

557773

Brand

BD Pharmingen™

Name

Wiskott-Aldrich Syndrome Protein

Alias

WASP

Concentration

0.25 mg/ml

Size

0.1mg

RegulatoryStatus

RUO

Clone

5A5

Format

Purified

Isotype

IgG_2a, κ

W.B. Positive Control

none

Molecular Weight

60 kDa

Immunogen

Human WASP Recombinant Protein

Strain Host

BALB/c

Reactivity

QC Testing : Human

Applications

Fluorescence microscopy	- Reported
Immunoprecipitation	- Reported
Intracellular staining (flow cytometry)	- Reported
Western Blot	- Routinely Tested