PION Proteins

Protein Information

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 patients¿ 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.

Synonyms

A530088I07Rik; gamma-secretase activating protein; gamma-secretase-activating protein; Gamma-secretase-activating protein 16 kDa C-terminal form; GSAP; GSAP-16K; pigeon homolog; Pion; Protein pigeon homolog; tcag7.1314