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Product Name WASP Chinese Name 湿疹血小板减少伴免疫缺陷综合征相关蛋白抗体 Alias Eczema thrombocytopenia; IMD2; SCNX; THC; THC1; Thrombocytopenia 1 (X linked); U42471; Was; WASp; WASP_HUMAN; Wiskott Aldrich syndrome (eczema thrombocytopenia); Wiskott Aldrich syndrome; Wiskott Aldrich syndrome protein; Wiskott-Aldrich syndrome protein. Research Area Cell biology immunology Cell differentiation Cytoskeleton Immunogen Species Rabbit Clonality Polyclonal React Species Human, Mouse, (predicted: Rat, Cow, Rabbit, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 53kDa Cellular localization cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human WASP: 101-200/502 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail The Wiskott-Aldrich syndrome (WAS) is a disorder that results from a monogenic defect that has been mapped to the short arm of the X chromosome. WAS is characterized by thrombocytopenia, eczema, defects in cell-mediated and humoral immunity and a propensity for lymphoproliferative disease. The gene that is mutated in the syndrome encodes a proline-rich protein of unknown function designated WAS protein (WASP). A clue to WASP function came from the observation that T cells from affected males had an irregular cellular morphology and a disarrayed cytoskeleton suggesting the involvement of WASP in cytoskeletal organization. Close examination of the WASP sequence revealed a putative Cdc42/Rac interacting domain, homologous with those found in PAK65 and ACK. Subsequent investigation has shown WASP to be a true downstream effector of Cdc42.
Function:
Effector protein for Rho-type GTPases, providing a link with the Arp2/3 complex that regulates the structure and dynamics of the actin cytoskeleton. Important for efficient actin polymerization. Possible regulator of lymphocyte and platelet function.
Subunit:
Interacts with NCK1 (via SH3 domains). Interacts with CDC42, RAC, NCK, HCK, FYN, SRC kinase FGR, BTK, ABL1, PSTPIP1, WIP, and to the p85 subunit of PLC-gamma. Binds the Arp2/3 complex. Interacts (via C-terminus) with ALDOA. Interacts with E.coli effector protein EspF(U).
Subcellular Location:
Cytoplasm; cytoskeleton.
Tissue Specificity:
Expressed predominantly in the thymus. Also found, to a much lesser extent, in the spleen
Post-translational modifications:
Phosphorylated at Tyr-291 by FYN and HCK, inducing WAS effector activity after TCR engagement. Phosphorylation at Tyr-291 enhances WAS activity in promoting actin polymerization and filopodia formation.
DISEASE:
Defects in WAS are the cause of Wiskott-Aldrich syndrome (WAS) [MIM:301000]; also known as eczema-thrombocytopenia-immunodeficiency syndrome.
WAS is an X-linked recessive immunodeficiency characterized by eczema, thrombocytopenia, recurrent infections, and bloody diarrhea. Death usually occurs before age 10. Defects in WAS are the cause of thrombocytopenia type 1 (THC1) [MIM:313900].
Thrombocytopenia is defined by a decrease in the number of platelets in circulating blood, resulting in the potential for increased bleeding and decreased ability for clotting.
Defects in WAS are a cause of neutropenia severe congenital X-linked (XLN) [MIM:300299]. XLN is an immunodeficiency syndrome characterized by recurrent major bacterial infections, severe congenital neutropenia, and monocytopenia.
Similarity:
Contains 1 CRIB domain.
Contains 1 WH1 domain.
Contains 1 WH2 domain.
SWISS:
P42768
Gene ID:
7454
Database links:Entrez Gene: 7454 Human
Entrez Gene: 22376 Mouse
Omim: 300392 Human
SwissProt: P42768 Human
SwissProt: P70315 Mouse
Unigene: 2157 Human
Product Picture Sample:
Jurkat(Human) Cell Lysate at 30 ug
Raji(Human) Cell Lysate at 30 ug
Primary: Anti-WASP (SL13681R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 53 kD
Observed band size: 53 kD
Sample:
U937(Human) Cell Lysate at 30 ug
Primary: Anti-WASP (SL13681R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 53 kD
Observed band size: 53 kD
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