2uuv: 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 ... [[http://ispc.weizmann.ac.il/pmbin/getpm?17562315 (full description)]]
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==
2UUV 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=2UUV OCA]].  
2UUV 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=2UUV OCA].  


==Reference==
==Reference==
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[[Category: transferase]]
[[Category: transferase]]


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