NP_059135
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
gamma-secretase-activating protein
NCBI Official Synonym Full Names
gamma-secretase activating protein
NCBI Official Synonym Symbols
NCBI Protein Information
gamma-secretase-activating protein; pigeon homolog; protein pigeon homolog
UniProt Protein Name
Gamma-secretase-activating protein
UniProt Synonym Protein Names
Protein pigeon homologGamma-secretase-activating protein 16 kDa C-terminal form; GSAP-16K
UniProt Synonym Gene Names
UniProt Entry Name
GSAP_HUMAN
NCBI Summary for PION
Accumulation of neurotoxic amyloid-beta is a major hallmark of Alzheimer disease (AD; MIM 104300). Formation of amyloid-beta is catalyzed by gamma-secretase (see PSEN1; MIM 104311), a protease with numerous substrates. PION, or GSAP, selectively increases amyloid-beta production through a mechanism involving its interaction with both gamma-secretase and its substrate, the amyloid-beta precursor protein (APP; MIM 104760) C-terminal fragment (APP-CTF) (He et al., 2010 [PubMed 20811458]).[supplied by OMIM, Nov 2010]
UniProt Comments for PION
GSAP: 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. Belongs to the GSAP family. 4 isoforms of the human protein are produced by alternative splicing.
Protein type: Activator
Chromosomal Location of Human Ortholog: 7q11.23
Cellular Component: trans-Golgi network
Molecular Function: beta-amyloid binding
Biological Process: regulation of proteolysis
Research Articles on PION
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Products associated with anti-PION antibody
Diseases associated with anti-PION antibody
Organs/Tissues associated with anti-PION antibody
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