2uuu: Difference between revisions
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==Overview== | ==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 .. | 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== | ==About this Structure== | ||
2UUU is a | 2UUU is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Dictyostelium_discoideum Dictyostelium discoideum] with FAD and PL3 as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Alkylglycerone-phosphate_synthase Alkylglycerone-phosphate synthase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.5.1.26 2.5.1.26] Structure known Active Site: AC1. Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=2UUU OCA]. | ||
==Reference== | ==Reference== | ||
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[[Category: transferase]] | [[Category: transferase]] | ||
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Revision as of 11:08, 5 November 2007
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ALKYLDIHYDROXYACETONEPHOSPHATE SYNTHASE IN P212121
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
2UUU is a Single protein structure of sequence from Dictyostelium discoideum with FAD and PL3 as ligands. Active as Alkylglycerone-phosphate synthase, with EC number 2.5.1.26 Structure known Active Site: AC1. 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
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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.
- FAD
- PL3
- Biosynthesis of phospholipids
- Fad
- Flavin
- Flavoprotein
- Lipid synthesis
- Peroxisomal disorder
- Peroxisome
- Plasmalogens
- Rhizomelic chondrodysplasia punctata
- Transferase
