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1y4j

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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'''
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===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.
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(as it appears on PubMed at http://www.pubmed.gov), where 15687489 is the PubMed ID number.
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{{ABSTRACT_PUBMED_15687489}}
==About this Structure==
==About this Structure==
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[[Category: Multiple sulfatase deficiency]]
[[Category: Multiple sulfatase deficiency]]
[[Category: Sulfatase]]
[[Category: Sulfatase]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Jul 28 02:33:46 2008''

Revision as of 23:33, 27 July 2008

Template:STRUCTURE 1y4j

Crystal structure of the paralogue of the human formylglycine generating enzyme

Template:ABSTRACT PUBMED 15687489

About this Structure

1Y4J is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Crystal structure of human pFGE, the paralog of the Calpha-formylglycine-generating enzyme., Dickmanns A, Schmidt B, Rudolph MG, Mariappan M, Dierks T, von Figura K, Ficner R, J Biol Chem. 2005 Apr 15;280(15):15180-7. Epub 2005 Feb 1. PMID:15687489

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