SUMF2 binds SUMF1, inhibiting SUMF1-mediated activation of arylsulfatases

Stable Identifier
Reaction [binding]
Homo sapiens
Locations in the PathwayBrowser
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout
Sulfatase-modifying factor 2 (SUMF2, also called C-alpha-formylglycine-generating enzyme 2, pFGE) is the paralogue of SUMF1. While SUMF1 can modify a critical residue on arylsulfatases to confer activity to them, SUMF2 lacks this ability (Mariappan et al. 2005) and instead, SUMF2 can inhibit the action of SUMF1 by dimerising with it (Zito et al. 2005, Fraldi et al. 2008, Zito et al. 2016).
Literature References
PubMed ID Title Journal Year
15708861 Expression, localization, structural, and functional characterization of pFGE, the paralog of the Calpha-formylglycine-generating enzyme

Balleininger, M, Eiselt, N, Preusser-Kunze, A, Mariappan, M, Gande, SL, von Figura, K, Wenzel, D, Dierks, T, Schmidt, B

J Biol Chem 2005
18508857 Multistep, sequential control of the trafficking and function of the multiple sulfatase deficiency gene product, SUMF1 by PDI, ERGIC-53 and ERp44

Cozzolino, M, Sitia, R, Monti, M, Fraldi, A, Cosma, MP, Zito, E, Spampanato, C, Pucci, P, Lombardi, A, Ballabio, A, Annunziata, F

Hum Mol Genet 2008
27909074 Sulfatase activities are regulated by the interaction of the sulfatase-modifying factor 1 with SUMF2

Pepe, S, Annunziata, I, Fraldi, A, Kobinger, G, Di Natale, P, Cosma, MP, Zito, E, Ballabio, A

EMBO Rep 2016
15962010 Sulphatase activities are regulated by the interaction of sulphatase-modifying factor 1 with SUMF2

Fraldi, A, Pepe, S, Kobinger, G, Ballabio, A, Annunziata, I, Cosma, MP, Di Natale, P, Zito, E

EMBO Rep 2005
Orthologous Events
Cite Us!