Code
BT-MCA2067
Host
Mouse
Isotype
Mouse IgG2a
Size
100μL,50μL
Clonality
Monoclonal
Immunogen
Purified recombinant fragment of human WAS (AA: 57-170) expressed in E. Coli.
Formulation
Purified antibody in PBS with 0.05% sodium azide.
Storage
Store at 4°C short term. Aliquot and store at -20°C long term.
Background
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 actin cytoskeleton. The presence of a number of different motifs suggests that they are regulated by a number of different stimuli, and interact with multiple proteins. Recent studies have demonstrated that these proteins, directly or indirectly, associate with the small GTPase, Cdc42, known to regulate formation of actin filaments, and the cytoskeletal organizing complex, Arp2/3. Wiskott-Aldrich syndrome is a rare, inherited, X-linked, recessive disease characterized by immune dysregulation and microthrombocytopenia, and is caused by mutations in the WAS gene. The WAS gene product is a cytoplasmic protein, expressed exclusively in hematopoietic cells, which show signalling and cytoskeletal abnormalities in WAS patients. A transcript variant arising 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.
Uniprot accession
Molecular weight
53kDa
Gene ID
7454
Synonyms
THC;IMD2;SCNX;THC1;WASP
Research area
Signal Transduction
Target protein
WAS
