1hzd: Difference between revisions

<table><tr><td colspan='2'>[[1hzd]] is a 6 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=1HZD OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1HZD 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]] 2.2&#8491;</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=1hzd FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1hzd OCA], [https://pdbe.org/1hzd PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1hzd RCSB], [https://www.ebi.ac.uk/pdbsum/1hzd PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1hzd ProSAT], [https://www.topsan.org/Proteins/RSGI/1hzd TOPSAN]</span></td></tr>

[https://www.uniprot.org/uniprot/AUHM_HUMAN AUHM_HUMAN] Defects in AUH are the cause of 3-methylglutaconic aciduria type 1 (MGA1) [MIM:[https://omim.org/entry/250950 250950]. MGA1 is an inborn error of leucine metabolism. It leads to an autosomal recessive syndrome with variable clinical phenotype, ranging from delayed speech development to severe psychomotor retardation, coma, failure to thrive, metabolic acidosis and dystonia. MGA1 can be distinguished from other forms of MGA by the pattern of metabolite excretion: 3-methylglutaconic acid levels are higher than those detected in other forms, whereas methylglutaric acid levels are usually only slightly elevated, and there is a high level of 3-hydroxyisovaleric acid excretion (not present in other MGA forms).<ref>PMID:12434311</ref> <ref>PMID:12655555</ref>  

[https://www.uniprot.org/uniprot/AUHM_HUMAN AUHM_HUMAN] Catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs.<ref>PMID:7892223</ref> <ref>PMID:12434311</ref> <ref>PMID:11738050</ref> <ref>PMID:12655555</ref>  

[[Category: Homo sapiens]]

[[Category: Fukai S]]

[[Category: Kurimoto K]]

[[Category: Muto Y]]

[[Category: Nureki O]]

[[Category: Yokoyama S]]