Anti-IKBKAP (Elongator Complex Protein 1, ELP1, IkappaB Kinase Complex-associated Protein, IKK Compl

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. Applications: Suitable for use in Immunofluorescence, ELISA, Western Blot and Immunocytochemistry. Other applications not tested. Recommended Dilution: Western Blot: 0.5-1ug/ml, Immunocytochemistry: 1ug/ml, Immunofluorescence: 20ug/ml, Optimal dilutions to be determined by the researcher. Positive Control: A20 Cell Lysate, Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.