|Application ||WB, IHC-P, IF, E|
|Other Accession||NP_059135, 148612805|
|Reactivity||Human, Mouse, Rat|
|Calculated MW||97802 Da|
|Application Notes||PION antibody can be used for detection of PION by Western blot at 0.25 µg/mL. Antibody can also be used for immunohistochemistry starting at 5 µg/mL. For immunofluorescence start at 20 µg/mL.|
|Target/Specificity||PION; Multiple isoforms of PION are known to exist. PION antibody is predicted to not cross-react with other F-box protein family members.|
|Reconstitution & Storage||PION antibody can be stored at 4℃ for three months and -20℃, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.|
|Precautions||PION Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Regulator of gamma-secretase activity, which specifically activates the production of beta-amyloid protein (beta-amyloid protein 40 and beta-amyloid protein 42), without affecting the cleavage of other gamma-secretase targets such has Notch. The gamma-secretase complex is an endoprotease complex that catalyzes the intramembrane cleavage of integral membrane proteins such as Notch receptors and APP (beta-amyloid precursor protein). Specifically promotes the gamma-cleavage of APP CTF-alpha (also named APP-CTF) by the gamma-secretase complex to generate beta- amyloid, while it reduces the epsilon-cleavage of APP CTF-alpha, leading to a low production of AICD.|
|Cellular Location||Golgi apparatus, trans-Golgi network|
|Tissue Location||Widely expressed.|
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Provided below are standard protocols that you may find useful for product applications.
PION Antibody: Accumulation of the amyloid-beta peptide (Abeta) in the cerebral cortex is a critical event in the pathogenesis of Alzheimer's disease. The beta-amyloid protein precursor (APP) is cleaved by one of two beta-secretases (BACE and BACE2), producing a soluble derivative of the protein and a membrane anchored 99 -amino acid carboxy-terminal fragment (C99). The C99 fragment serves as substrate for gamma-secretase to generate the 4 kDa amyloid-beta peptide (Abeta), which is deposited in the Alzheimer's disease patient's brains. PION, or GSAP, selectively increases amyloid-beta production through a mechanism involving its interaction with both gamma-secretase and the APP C-terminal fragment, suggesting that PION may be a potential therapeutic target for the treatment of Alzheimer's disease.
Ponte P, Gonzalez-DeWhitt P, Schilling J, et al. A new A4 amyloid mRNA contains a domain homologous to serine proteinase inhibitors. Nature1988; 331:525-77.
Selkoe DJ. Cell biology of the amyloid beta-protein precursor and the mechanism of Alzheimer's disease. Annu. Rev. Cell Biol.1994; 10:373-403.
He G, Luo W, Li P, et al. Gamma-secretase activating protein is a therapeutic target for Alzheimer’s diease. Nature2010; 467:95-9.
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