8hwl: Difference between revisions
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==Human Pyruvate Carboxylase== | |||
<StructureSection load='8hwl' size='340' side='right'caption='[[8hwl]], [[Resolution|resolution]] 5.63Å' scene=''> | |||
== Structural highlights == | |||
<table><tr><td colspan='2'>[[8hwl]] is a 4 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=8HWL OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8HWL FirstGlance]. <br> | |||
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 5.63Å</td></tr> | |||
[[Category: | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=BTI:5-(HEXAHYDRO-2-OXO-1H-THIENO[3,4-D]IMIDAZOL-6-YL)PENTANAL'>BTI</scene></td></tr> | ||
[[Category: Liu | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=8hwl FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8hwl OCA], [https://pdbe.org/8hwl PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8hwl RCSB], [https://www.ebi.ac.uk/pdbsum/8hwl PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8hwl ProSAT]</span></td></tr> | ||
[[Category: Su | </table> | ||
== Disease == | |||
[https://www.uniprot.org/uniprot/PYC_HUMAN PYC_HUMAN] Defects in PC are the cause of pyruvate carboxylase deficiency (PC deficiency) [MIM:[https://omim.org/entry/266150 266150]. PC deficiency leads to lactic acidosis, mental retardation and death. It occurs in three forms: mild or type A, severe neonatal or type B, and a very mild lacticacidemia. | |||
== Function == | |||
[https://www.uniprot.org/uniprot/PYC_HUMAN PYC_HUMAN] Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate. | |||
__TOC__ | |||
</StructureSection> | |||
[[Category: Homo sapiens]] | |||
[[Category: Large Structures]] | |||
[[Category: Liu DS]] | |||
[[Category: Su JY]] | |||
