IKAP was initially identified as a scaffold protein of the IkB kinase complex that could bind to IKKa, IKKb, NF-kB, and the NF-kB-inducing kinase (NIK), although later evidence has cast doubt on this. More recent reports show that mutations in IKAP such as a frameshift leading to a truncated protein or a missense mutation that leads to defective phosphorylation are responsible for the autosomal recessive genetic disease familial dysautonomia (FD). Reports indicating that it forms part of the RNA polymerase II transcription elongation complex suggest that this disease may be due to compromised transcription elongation. More recently, it was shown that IKAP associates with c-Jun N-terminal kinase (JNK) and could specifically enhance JNK activation induced by the upstream JNK activators MEKK1 and ASK1, indicating another possible cause for FD. At least two isoforms of IKAP are known two exist.
应用类型
ELISA,IF Microscopy,Western Blot,
免疫原
Anti-IKAP antibody was prepared from whole rabbit serum produced by repeated immunizations with a 16 amino acid synthetic peptide from near the C-terminus of human IKAP.
来源宿主
Rabbit
反应性
H. sapiens (Human); Mus musculus (Mouse)
保存建议
Store vial at -20° C prior to opening. Aliquot contents and freeze at -20° C or below for extended storage. Avoid cycles of freezing and thawing. Centrifuge product if not completely clear after standing at room temperature. This product is stable for several weeks at 4° C as an undiluted liquid. Dilute only prior to immediate use.
