Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme
Dierks T, Dickmanns A, Preusser-Kunze A, Schmidt B, Mariappan M, Figura von K, Ficner R, Rudolph MG (2005)
CELL 121(4): 541-552.
Zeitschriftenaufsatz
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Autor*in
Dierks, ThomasUniBi;
Dickmanns, A;
Preusser-Kunze, A;
Schmidt, B;
Mariappan, M;
Figura von, K;
Ficner, R;
Rudolph, MG
Einrichtung
Abstract / Bemerkung
Sulfatases are enzymes essential for degradation and remodeling of sulfate esters. Formylglycine (FGly), the key catalytic residue in the active site, is unique to sulfatases. In higher eukaryotes, FGly is generated from a cysteine precursor by the FGly-generating enzyme (FGE). Inactivity of FGE results in multiple sulfatase deficiency (MSD), a fatal autosomal recessive syndrome. Based on the crystal structure, we report that FGE is a single-domain monomer with a surprising paucity of secondary structure and adopts a unique fold. The effect of all 18 missense mutations found in MSD patients is explained by the FGE structure, providing a molecular basis of MSD. The catalytic mechanism of FGly generation was elucidated by six high-resolution structures of FGE in different redox environments. The structures allow formulation of a novel oxygenase mechanism whereby FGE utilizes molecular oxygen to generate FGly via a cysteine sulfenic acid intermediate.
Erscheinungsjahr
2005
Zeitschriftentitel
CELL
Band
121
Ausgabe
4
Seite(n)
541-552
ISSN
0092-8674
Page URI
https://pub.uni-bielefeld.de/record/2350692
Zitieren
Dierks T, Dickmanns A, Preusser-Kunze A, et al. Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme. CELL. 2005;121(4):541-552.
Dierks, T., Dickmanns, A., Preusser-Kunze, A., Schmidt, B., Mariappan, M., Figura von, K., Ficner, R., et al. (2005). Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme. CELL, 121(4), 541-552. https://doi.org/10.1016/j.cell.2005.03.001
Dierks, Thomas, Dickmanns, A, Preusser-Kunze, A, Schmidt, B, Mariappan, M, Figura von, K, Ficner, R, and Rudolph, MG. 2005. “Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme”. CELL 121 (4): 541-552.
Dierks, T., Dickmanns, A., Preusser-Kunze, A., Schmidt, B., Mariappan, M., Figura von, K., Ficner, R., and Rudolph, M. G. (2005). Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme. CELL 121, 541-552.
Dierks, T., et al., 2005. Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme. CELL, 121(4), p 541-552.
T. Dierks, et al., “Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme”, CELL, vol. 121, 2005, pp. 541-552.
Dierks, T., Dickmanns, A., Preusser-Kunze, A., Schmidt, B., Mariappan, M., Figura von, K., Ficner, R., Rudolph, M.G.: Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme. CELL. 121, 541-552 (2005).
Dierks, Thomas, Dickmanns, A, Preusser-Kunze, A, Schmidt, B, Mariappan, M, Figura von, K, Ficner, R, and Rudolph, MG. “Molecular basis for multiple sulfatase deficiency and mechanism for formylglycine generation of the human formylglycine-generating enzyme”. CELL 121.4 (2005): 541-552.
Daten bereitgestellt von European Bioinformatics Institute (EBI)
UNIPROT
2 Einträge gefunden, die diesen Artikel zitieren
PDB
6 Einträge gefunden, die diesen Artikel zitieren
x-ray diffraction (PDB: 1y1j)
Protein structure name: human formylglycine generating enzyme, sulfonic acid/desulfurated form
Public wwPDB file in PDB format
Protein structure name: human formylglycine generating enzyme, sulfonic acid/desulfurated form
Public wwPDB file in PDB format
x-ray diffraction (PDB: 1y1i)
Protein structure name: hyuman formylglycine generating enzyme, reduced form
Public wwPDB file in PDB format
Protein structure name: hyuman formylglycine generating enzyme, reduced form
Public wwPDB file in PDB format
x-ray diffraction (PDB: 1y1g)
Protein structure name: human formylglycine generating enzyme, double sulfonic acid form
Public wwPDB file in PDB format
Protein structure name: human formylglycine generating enzyme, double sulfonic acid form
Public wwPDB file in PDB format
x-ray diffraction (PDB: 1y1e)
Protein structure name: human formylglycine generating enzyme
Public wwPDB file in PDB format
Protein structure name: human formylglycine generating enzyme
Public wwPDB file in PDB format
x-ray diffraction (PDB: 1y1h)
Protein structure name: human formylglycine generating enzyme, oxidised cys refined as hydroperoxide
Public wwPDB file in PDB format
Protein structure name: human formylglycine generating enzyme, oxidised cys refined as hydroperoxide
Public wwPDB file in PDB format
x-ray diffraction (PDB: 1y1f)
Protein structure name: human formylglycine generating enzyme with cysteine sulfenic acid
Public wwPDB file in PDB format
Protein structure name: human formylglycine generating enzyme with cysteine sulfenic acid
Public wwPDB file in PDB format
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