Formylglycine-generating enzyme

Definition:

Oxidase that catalyzes the conversion of cysteine to 3-oxoalanine on target proteins, using molecular oxygen and an unidentified reducing agent. 3-oxoalanine modification, which is also named formylglycine (fGly), occurs in the maturation of arylsulfatases and some alkaline phosphatases that use the hydrated form of 3-oxoalanine as a catalytic nucleophile. Known substrates include GALNS, ARSA, STS and ARSE.

References:

[1]. Andrea Preusser-Kunze, et al. Molecular characterization of the human Calpha-formylglycine-generating enzyme. J Biol Chem. 2005 Apr 15;280(15):14900-10. [Content Brief]

[2]. Dirk Roeser, et al. A general binding mechanism for all human sulfatases by the formylglycine-generating enzyme. Proc Natl Acad Sci U S A. 2006 Jan 3;103(1):81-6. [Content Brief]

[3]. Patrick G Holder, et al. Reconstitution of Formylglycine-generating Enzyme with Copper(II) for Aldehyde Tag Conversion. J Biol Chem. 2015 Jun 19;290(25):15730-15745. [Content Brief]

[4]. Maria Pia Cosma, et al. The multiple sulfatase deficiency gene encodes an essential and limiting factor for the activity of sulfatases. Cell. 2003 May 16;113(4):445-56. [Content Brief]

[5]. Thomas Dierks, et al. Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme. Cell. 2005 May 20;121(4):541-552. [Content Brief]

[6]. Lars Schlotawa, et al. SUMF1 mutations affecting stability and activity of formylglycine generating enzyme predict clinical outcome in multiple sulfatase deficiency. Eur J Hum Genet. 2011 Mar;19(3):253-61. [Content Brief]

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