1now: Difference between revisions

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-[[Image:1now.png|left|200px]]
-{{STRUCTURE_1now|  PDB=1now  |  SCENE=  }}
+==Human lysosomal beta-hexosaminidase isoform B in complex with (2R,3R,4S,5R)-2-Acetamido-3,4-Dihydroxy-5-Hydroxymethyl-Piperidinium Chloride (GalNAc-isofagomine)==
+<StructureSection load='1now' size='340' side='right'caption='[[1now]], [[Resolution|resolution]] 2.20&Aring;' scene=''>
-===Human lysosomal beta-hexosaminidase isoform B in complex with (2R,3R,4S,5R)-2-Acetamido-3,4-Dihydroxy-5-Hydroxymethyl-Piperidinium Chloride (GalNAc-isofagomine)===
+== Structural highlights ==
+<table><tr><td colspan='2'>[[1now]] 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=1NOW OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1NOW FirstGlance]. <br>
-{{ABSTRACT_PUBMED_12662933}}
+</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='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=IFG:(2R,3R,4S,5R)-2-ACETAMIDO-3,4-DIHYDROXY-5-HYDROXYMETHYL-PIPERIDINE'>IFG</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
-==About this Structure==
+<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1now FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1now OCA], [https://pdbe.org/1now PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1now RCSB], [https://www.ebi.ac.uk/pdbsum/1now PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1now ProSAT]</span></td></tr>
-[[1now]] is a 2 chain structure of [[Beta-Hexosaminidase]] with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1NOW OCA].
+</table>
+== Disease ==
+[https://www.uniprot.org/uniprot/HEXB_HUMAN HEXB_HUMAN] Defects in HEXB are the cause of GM2-gangliosidosis type 2 (GM2G2) [MIM:[https://omim.org/entry/268800 268800]; also known as Sandhoff disease. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G2 is clinically indistinguishable from GM2-gangliosidosis type 1, presenting startle reactions, early blindness, progressive motor and mental deterioration, macrocephaly and cherry-red spots on the macula.<ref>PMID:1720305</ref> <ref>PMID:1531140</ref> <ref>PMID:8357844</ref> <ref>PMID:7626071</ref> <ref>PMID:7557963</ref> <ref>PMID:7633435</ref> <ref>PMID:8950198</ref> <ref>PMID:9401004</ref> <ref>PMID:9856491</ref> <ref>PMID:9694901</ref>
+== Function ==
+[https://www.uniprot.org/uniprot/HEXB_HUMAN HEXB_HUMAN] Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.
+== Evolutionary Conservation ==
+[[Image:Consurf_key_small.gif|200px|right]]
+Check<jmol>
+  <jmolCheckbox>
+    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/no/1now_consurf.spt"</scriptWhenChecked>
+    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
+    <text>to colour the structure by Evolutionary Conservation</text>
+  </jmolCheckbox>
+</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=1now ConSurf].
+<div style="clear:both"></div>
 ==See Also==
 *[[Beta-Hexosaminidase|Beta-Hexosaminidase]]
+*[[Beta-Hexosaminidase 3D structures|Beta-Hexosaminidase 3D structures]]
-==Reference==
+*[[Beta-N-acetylhexosaminidase 3D structures|Beta-N-acetylhexosaminidase 3D structures]]
-<ref group="xtra">PMID:012662933</ref><references group="xtra"/>
+== References ==
-[[Category: Beta-N-acetylhexosaminidase]]
+<references/>
+__TOC__
+</StructureSection>
 [[Category: Homo sapiens]]
-[[Category: Cherney, M M.]]
+[[Category: Large Structures]]
-[[Category: James, M N.G.]]
+[[Category: Cherney MM]]
-[[Category: Knapp, S.]]
+[[Category: James MNG]]
-[[Category: Mahuran, D J.]]
+[[Category: Knapp S]]
-[[Category: Mark, B L.]]
+[[Category: Mahuran DJ]]
-[[Category: Zhao, D.]]
+[[Category: Mark BL]]
-[[Category: Family 20 glycosidase]]
+[[Category: Zhao D]]
-[[Category: Homodimer]]
-[[Category: Hydrolase]]