Anti-POMP Antibody Picoband® Fluoro647 Conjugated

Proteasome maturation protein is a protein that in humans is encoded by the POMP gene. The protein encoded by this gene is a molecular chaperone that binds 20S preproteasome components and is essential for 20S proteasome formation. The 20S proteasome is the proteolytically active component of the 26S proteasome complex. The encoded protein is degraded before the maturation of the 20S proteasome is complete. A variant in the 5' UTR of this gene has been associated with KLICK syndrome, a rare skin disorder.

NACHT, LRR and PYD domains-containing protein 2; Nucleotide-binding site protein 1; PYRIN domain and NACHT domain-containing protein 1; PYRIN-containing APAF1-like protein 2; NLRP2; NALP2; NBS1; PAN1; PYPAF2

Suppresses TNF- and CD40-induced NFKB1 activity at the level of the IKK complex, by inhibiting NFKBIA degradation induced by TNF. When associated with PYCARD, activates CASP1, leading to the secretion of mature proinflammatory cytokine IL1B. May be a component of the inflammasome, a protein complex which also includes PYCARD, CARD8 and CASP1 and whose function would be the activation of proinflammatory caspases.

1. Brehm, A., Liu, Y., Sheikh, A., Marrero, B., Omoyinmi, E., Zhou, Q., Montealegre, G., Biancotto, A., Reinhardt, A., Almeida de Jesus, A., Pelletier, M., Tsai, W. L., and 31 others. Additive loss-of-function proteasome subunit mutations in CANDLE/PRAAS patients promote type I IFN production. J. Clin. Invest. 125: 4196-4211, 2015. Note: Erratum: J. Clin. Invest. 126: 795 only, 2016. 2. Chondrogianni, N., Gonos, E. S. Overexpression of hUMP1/POMP proteasome accessory protein enhances proteasome-mediated antioxidant defence. Exp. Gerontol. 42: 899-903, 2007. 3. Dahlqvist, J., Klar, J., Tiwari, N., Schuster, J., Torma, H., Badhai, J., Pujol, R., van Steensel, M. A. M., Brinkhuizen, T., Gijezen, L., Chaves, A., Tadini, G., Vahlquist, A., Dahl, N. A single-nucleotide deletion in the POMP 5-prime UTR causes a transcriptional switch and altered epidermal proteasome distribution in KLICK genodermatosis. Am. J. Hum. Genet. 86: 596-603, 2010. Note: Erratum: Am. J. Hum. Genet. 86: 655 only, 2010.