MBTPS1,2 cleaves CREB3L3 to CREB3L3(1-?) and CREB3L3(?-461)

Stable Identifier
Reaction [transition]
Homo sapiens
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Cyclic AMP-responsive element-binding protein 3-like protein 3 (CREB3L3) is a transcription factor that is highly and selectively expressed in liver and small intestine. This protein is produced as an ER-localized type II transmembrane glycoprotein that is converted into the mature nuclear form (the N-terminal portion) by sequence specific proteases, membrane-bound transcription factor site-1 proteases 1 and 2 (MBTPS1 and 2) (Marschner et al. 2011, Oeffner et al. 2009). CREB3L3 is a downstream target gene of hepatocyte nuclear factor 4a (HNF-4a), which plays a critical role in hepatocyte differentiation and liver function. Ablation of HNF-4a abolished CREB-H mRNA expression in the liver, but not in the small intestine, suggesting an essential role of HNF-4a in hepatic CREB-H expression.

Literature References
PubMed ID Title Journal Year
21719679 A key enzyme in the biogenesis of lysosomes is a protease that regulates cholesterol metabolism

Braulke, T, Kollmann, K, Schweizer, M, Marschner, K, Pohl, S

Science 2011
19361614 IFAP syndrome is caused by deficiency in MBTPS2, an intramembrane zinc metalloprotease essential for cholesterol homeostasis and ER stress response

Fischer, G, Redler, S, Romero-Gomez, J, Oeffner, F, Vera-CasaƱo, A, Konig, A, Weirich, C, Bornholdt, D, Happle, R, Grzeschik, KH, Boente, Mdel C, Neidel, U, Betz, RC, Salhi, A

Am. J. Hum. Genet. 2009
Catalyst Activity

metalloendopeptidase activity of MBTPS1,2 [endoplasmic reticulum membrane]

Inferred From
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