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| {{STRUCTURE_1y4j| PDB=1y4j | SCENE= }} | | {{STRUCTURE_1y4j| PDB=1y4j | SCENE= }} |
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| '''Crystal structure of the paralogue of the human formylglycine generating enzyme'''
| | ===Crystal structure of the paralogue of the human formylglycine generating enzyme=== |
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| ==Overview==
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| In eukaryotes, sulfate esters are degraded by sulfatases, which possess a unique Calpha-formylglycine residue in their active site. The defect in post-translational formation of the Calpha-formylglycine residue causes a severe lysosomal storage disorder in humans. Recently, FGE (formylglycine-generating enzyme) has been identified as the protein required for this specific modification. Using sequence comparisons, a protein homologous to FGE was found and denoted pFGE (paralog of FGE). pFGE binds a sulfatase-derived peptide bearing the FGE recognition motif, but it lacks formylglycine-generating activity. Both proteins belong to a large family of pro- and eukaryotic proteins containing the DUF323 domain, a formylglycine-generating enzyme domain of unknown three-dimensional structure. We have crystallized the glycosylated human pFGE and determined its crystal structure at a resolution of 1.86 A. The structure reveals a novel fold, which we denote the FGE fold and which therefore serves as a paradigm for the DUF323 domain. It is characterized by an asymmetric partitioning of secondary structure elements and is stabilized by two calcium cations. A deep cleft on the surface of pFGE most likely represents the sulfatase polypeptide binding site. The asymmetric unit of the pFGE crystal contains a homodimer. The putative peptide binding site is buried between the monomers, indicating a biological significance of the dimer. The structure suggests the capability of pFGE to form a heterodimer with FGE.
| | The line below this paragraph, {{ABSTRACT_PUBMED_15687489}}, adds the Publication Abstract to the page |
| | (as it appears on PubMed at http://www.pubmed.gov), where 15687489 is the PubMed ID number. |
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| | {{ABSTRACT_PUBMED_15687489}} |
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| ==About this Structure== | | ==About this Structure== |
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| [[Category: Multiple sulfatase deficiency]] | | [[Category: Multiple sulfatase deficiency]] |
| [[Category: Sulfatase]] | | [[Category: Sulfatase]] |
| ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 15:52:17 2008'' | | |
| | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Jul 28 02:33:46 2008'' |