2UUV
alkyldihydroxyacetonephosphate synthase in P1
Summary for 2UUV
| Entry DOI | 10.2210/pdb2uuv/pdb |
| Related | 2UUU |
| Descriptor | ALKYLDIHYDROXYACETONEPHOSPHATE SYNTHASE, FLAVIN-ADENINE DINUCLEOTIDE, HEXADECAN-1-OL, ... (4 entities in total) |
| Functional Keywords | rhizomelic chondrodysplasia punctata, biosynthesis of phospholipids, flavoprotein, lipid synthesis, peroxisomal disorder, fad, flavin, peroxisome, transferase, plasmalogens |
| Biological source | DICTYOSTELIUM DISCOIDEUM (SLIME MOLD) |
| Cellular location | Peroxisome: O96759 |
| Total number of polymer chains | 4 |
| Total formula weight | 268288.46 |
| Authors | Razeto, A.,Mattiroli, F.,Carpanelli, E.,Aliverti, A.,Pandini, V.,Coda, A.,Mattevi, A. (deposition date: 2007-03-07, release date: 2007-06-26, Last modification date: 2024-05-08) |
| Primary citation | Razeto, A.,Mattiroli, F.,Carpanelli, E.,Aliverti, A.,Pandini, V.,Coda, A.,Mattevi, A. The Crucial Step in Ether Phospholipid Biosynthesis: Structural Basis of a Noncanonical Reaction Associated with a Peroxisomal Disorder. Structure, 15:683-, 2007 Cited by PubMed Abstract: 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. PubMed: 17562315DOI: 10.1016/J.STR.2007.04.009 PDB entries with the same primary citation |
| Experimental method | X-RAY DIFFRACTION (1.99 Å) |
Structure validation
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