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| [[Image:2uuv.gif|left|200px]] | | {{Seed}} |
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| {{STRUCTURE_2uuv| PDB=2uuv | SCENE= }} | | {{STRUCTURE_2uuv| PDB=2uuv | SCENE= }} |
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| '''ALKYLDIHYDROXYACETONEPHOSPHATE SYNTHASE IN P1'''
| | ===ALKYLDIHYDROXYACETONEPHOSPHATE SYNTHASE IN P1=== |
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| ==Overview==
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| Ether phospholipids are essential constituents of eukaryotic cell membranes. Rhizomelic chondrodysplasia punctata type 3 is a severe peroxisomal disorder caused by inborn deficiency of alkyldihydroxyacetonephosphate synthase (ADPS). The enzyme carries out the most characteristic step in ether phospholipid biosynthesis: formation of the ether bond. The crystal structure of ADPS from Dictyostelium discoideum shows a fatty-alcohol molecule bound in a narrow hydrophobic tunnel, specific for aliphatic chains of 16 carbons. Access to the tunnel is controlled by a flexible loop and a gating helix at the protein-membrane interface. Structural and mutagenesis investigations identify a cluster of hydrophilic catalytic residues, including an essential tyrosine, possibly involved in substrate proton abstraction, and the arginine that is mutated in ADPS-deficient patients. We propose that ether bond formation might be orchestrated through a covalent imine intermediate with the flavin, accounting for the noncanonical employment of a flavin cofactor in a nonredox reaction.
| | The line below this paragraph, {{ABSTRACT_PUBMED_17562315}}, adds the Publication Abstract to the page |
| | (as it appears on PubMed at http://www.pubmed.gov), where 17562315 is the PubMed ID number. |
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| | {{ABSTRACT_PUBMED_17562315}} |
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| ==About this Structure== | | ==About this Structure== |
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| [[Category: Rhizomelic chondrodysplasia punctata]] | | [[Category: Rhizomelic chondrodysplasia punctata]] |
| [[Category: Transferase]] | | [[Category: Transferase]] |
| ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun May 4 17:31:48 2008'' | | |
| | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Jul 28 17:35:53 2008'' |
