The PIK-related kinases include Atm, DNA-PKCS and mTOR. The Atm gene is mutated in the autosomal recessive disorder ataxia telangiectasia (AT) that is characterized by cerebellar degeneration and the appearance of dilated blood vessels in the conjunctivae of the eyes. AT cells are hypersensitive to ionizing radiation, impaired in mediating the inhibition of DNA synthesis and they display delays in p53 induction. DNA-PK is a heterotrimeric DNA binding enzyme that is composed of a large subunit, DNA-PKCS, and two smaller subunits collectively known as Ku. The loss of DNA-PK leads to defects in DSB repair and V(D)J recombination. mTOR, also known as FRAP, can autophosphorylate on serine and bind to rapamycin/FKBP. mTOR is also an upstream regulator of S6 kinase and has been implicated in the regulation of p27 and p21 expression. mTOR autophosphorylates at Ser2481 under translationally repressive conditions. Phosphorylation of mTOR at Ser2448 is mediated by p70S6 kinase. (Shipping Cost: €2

CD123/IL3RA [7G3]

Human CD123, the 70 kD IL-3 receptor chain (IL-3R α), is associated with the 120-140 kD β subunit. The β chain is shared with the receptors for interleukins IL-5 and GM-CSF. IL-3R α is expressed on hematopoietic progenitors and plays an important role in hematopoietic progenitor cell growth and differentiation. This antibody has been reported to block the binding of 125I-IL-3 to high and low affinity IL-3 receptors. In functional experiments, this antibody was found to inhibit acute myeloid leukemia cell proliferation, basophilhistamine release, endothelial cell-mediated IL-8 secretion, and neutrophil transmigration. At the Fifth HLDA Workshop, the human IL-3 receptor was designated CD123. This antibody reacts with plasmacytoid dendritic cells (PDC). PDC plays a crucial role in the initiation of of antiviral and antitumoral immune responses and produce type I interferons α and β. CD123 antibody is useful in diagnosis of Kikuchi disease, hyaline vascular type of Castleman disease, lupus, and primary cutaneous marginal zone B-cell lymphoma.

CD123/IL3RA [7G3]