PEN-2

PSENEN
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesPSENEN, MSTP064, PEN-2, PEN2, MDS033, presenilin enhancer gamma-secretase subunit, ACNINV2, presenilin enhancer, gamma-secretase subunit
External IDsOMIM: 607632; MGI: 1913590; HomoloGene: 11952; GeneCards: PSENEN; OMA:PSENEN - orthologs
Orthologs
SpeciesHumanMouse
Entrez

55851

66340

Ensembl

ENSG00000205155

ENSMUSG00000036835

UniProt

Q9NZ42

Q9CQR7

RefSeq (mRNA)

NM_172341
NM_001281532
NM_018468

NM_025498
NM_001364016
NM_001364017

RefSeq (protein)

NP_001268461
NP_758844

NP_079774
NP_001350945
NP_001350946

Location (UCSC)Chr 19: 35.75 – 35.75 MbChr 7: 30.26 – 30.26 Mb
PubMed search
Wikidata
View/Edit HumanView/Edit Mouse

PSENEN, formally PEN-2 (presenilin enhancer 2), is a protein that is a regulatory component of the gamma secretase complex, a protease complex responsible for proteolysis of transmembrane proteins such as the Notch protein and amyloid precursor protein (APP). The gamma secretase complex consists of PEN-2, APH-1, nicastrin, and the catalytic subunit presenilin. PEN-2 is a 101-amino acid integral membrane protein likely with a topology such that both the N-terminus and the C-terminus face first the lumen of the endoplasmic reticulum and later the extracellular environment. Biochemical studies have shown that a conserved sequence motif D-Y-L-S-F at the C-terminus, as well as the overall length of the C-terminal tail, is required for the formation of an active gamma secretase complex.

PEN-2 binds to metformin at therapeutic concentration and may be responsible for its AMPK-activating effects. PEN2-metformin in turn binds to ATP6AP1 to inhibit v-ATPase activity.