2cm0: Difference between revisions

<table><tr><td colspan='2'>[[2cm0]] is a 1 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=2CM0 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2CM0 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]] 1.9&#8491;</td></tr>

<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=BME:BETA-MERCAPTOETHANOL'>BME</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</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=2cm0 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2cm0 OCA], [https://pdbe.org/2cm0 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2cm0 RCSB], [https://www.ebi.ac.uk/pdbsum/2cm0 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2cm0 ProSAT]</span></td></tr>

[https://www.uniprot.org/uniprot/NGLY1_HUMAN NGLY1_HUMAN] Alacrimia-choreoathetosis-liver dysfunction syndrome. The disease is caused by mutations affecting the gene represented in this entry.

[https://www.uniprot.org/uniprot/NGLY1_HUMAN NGLY1_HUMAN] Specifically deglycosylates the denatured form of N-linked glycoproteins in the cytoplasm and assists their proteasome-mediated degradation. Cleaves the beta-aspartyl-glucosamine (GlcNAc) of the glycan and the amide side chain of Asn, converting Asn to Asp. Prefers proteins containing high-mannose over those bearing complex type oligosaccharides. Can recognize misfolded proteins in the endoplasmic reticulum that are exported to the cytosol to be destroyed and deglycosylate them, while it has no activity toward native proteins. Deglycosylation is a prerequisite for subsequent proteasome-mediated degradation of some, but not all, misfolded glycoproteins.<ref>PMID:14749736</ref> <ref>PMID:15358861</ref>  

[[Category: Homo sapiens]]

[[Category: Allen MD]]

[[Category: Buchberger A]]

[[Category: Bycroft M]]