2uuv | pdb_00002uuv
ALKYLDIHYDROXYACETONEPHOSPHATE SYNTHASE IN P1
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| 2uuv, resolution 1.99Å | |||||||||||||
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| Sites: | AC1 | ||||||||||||
| Ligands: | FAD, PL3 | ||||||||||||
| Activity: | Alkylglycerone-phosphate synthase, with EC number 2.5.1.26 | ||||||||||||
| Resources: | FirstGlance, OCA, PDBsum, RCSB | ||||||||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||||||||
Overview
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.
About this Structure
2UUV is a Single protein structure of sequence from Dictyostelium discoideum. Full crystallographic information is available from OCA.
Reference
The crucial step in ether phospholipid biosynthesis: structural basis of a noncanonical reaction associated with a peroxisomal disorder., Razeto A, Mattiroli F, Carpanelli E, Aliverti A, Pandini V, Coda A, Mattevi A, Structure. 2007 Jun;15(6):683-92. PMID:17562315
Page seeded by OCA on Mon Mar 31 05:04:49 2008
Proteopedia Page Contributors and Editors (what is this?)
- Pages with broken file links
- Alkylglycerone-phosphate synthase
- Dictyostelium discoideum
- Single protein
- Aliverti, A.
- Carpanelli, E.
- Coda, A.
- Mattevi, A.
- Mattiroli, F.
- Pandini, V.
- Razeto, A.
- Biosynthesis of phospholipid
- Fad
- Flavin
- Flavoprotein
- Lipid synthesis
- Peroxisomal disorder
- Peroxisome
- Plasmalogen
- Rhizomelic chondrodysplasia punctata
- Transferase
