6hg8: Difference between revisions

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-'''Unreleased structure'''
-The entry 6hg8 is ON HOLD  until Paper Publication
+==Crystal structure of the R460G disease-causing mutant of the human dihydrolipoamide dehydrogenase.==
+<StructureSection load='6hg8' size='340' side='right'caption='[[6hg8]], [[Resolution|resolution]] 1.76&Aring;' scene=''>
+== Structural highlights ==
+<table><tr><td colspan='2'>[[6hg8]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6HG8 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6HG8 FirstGlance]. <br>
+</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.76&#8491;</td></tr>
+<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=TRS:2-AMINO-2-HYDROXYMETHYL-PROPANE-1,3-DIOL'>TRS</scene></td></tr>
+<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=6hg8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6hg8 OCA], [https://pdbe.org/6hg8 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6hg8 RCSB], [https://www.ebi.ac.uk/pdbsum/6hg8 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6hg8 ProSAT]</span></td></tr>
+</table>
+== Disease ==
+[https://www.uniprot.org/uniprot/DLDH_HUMAN DLDH_HUMAN] Note=Defects in DLD are involved in the development of congenital infantile lactic acidosis.  Defects in DLD are a cause of maple syrup urine disease (MSUD) [MIM:[https://omim.org/entry/248600 248600]. MSUD is characterized by mental and physical retardation, feeding problems and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine, resulting from a block in oxidative decarboxylation.
+== Function ==
+[https://www.uniprot.org/uniprot/DLDH_HUMAN DLDH_HUMAN] Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
-Authors: Ambrus, A., Szabo, E., Weichsel, A., Bui, D., Wilk, P., Torocsik, B., Weiss, M.S., Montfort, W.R., Jordan, F., Adam-Vizi, V.
+==See Also==
+*[[Dihydrolipoamide dehydrogenase|Dihydrolipoamide dehydrogenase]]
-Description: Crystal structure of the R460G disease-causing mutant of the human dihydrolipoamide dehydrogenase.
+__TOC__
-[[Category: Unreleased Structures]]
+</StructureSection>
-[[Category: Jordan, F]]
+[[Category: Homo sapiens]]
-[[Category: Torocsik, B]]
+[[Category: Large Structures]]
-[[Category: Ambrus, A]]
+[[Category: Adam-Vizi V]]
-[[Category: Wilk, P]]
+[[Category: Ambrus A]]
-[[Category: Adam-Vizi, V]]
+[[Category: Bui D]]
-[[Category: Szabo, E]]
+[[Category: Jordan F]]
-[[Category: Montfort, W.R]]
+[[Category: Montfort WR]]
-[[Category: Weichsel, A]]
+[[Category: Szabo E]]
-[[Category: Weiss, M.S]]
+[[Category: Torocsik B]]
-[[Category: Bui, D]]
+[[Category: Weichsel A]]
+[[Category: Weiss MS]]
+[[Category: Wilk P]]