2etl

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[[Image:2etl.gif|left|200px]]
 
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==Crystal Structure of Ubiquitin Carboxy-terminal Hydrolase L1 (UCH-L1)==
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The line below this paragraph, containing "STRUCTURE_2etl", creates the "Structure Box" on the page.
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<StructureSection load='2etl' size='340' side='right'caption='[[2etl]], [[Resolution|resolution]] 2.40&Aring;' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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<table><tr><td colspan='2'>[[2etl]] 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=2ETL OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2ETL FirstGlance]. <br>
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or leave the SCENE parameter empty for the default display.
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.4&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene></td></tr>
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{{STRUCTURE_2etl| PDB=2etl | SCENE= }}
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2etl FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2etl OCA], [https://pdbe.org/2etl PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2etl RCSB], [https://www.ebi.ac.uk/pdbsum/2etl PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2etl ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/UCHL1_HUMAN UCHL1_HUMAN] Defects in UCHL1 are the cause of Parkinson disease type 5 (PARK5) [MIM:[https://omim.org/entry/613643 613643]; also known as Parkinson disease autosomal dominant 5. PARK5 is a complex neurodegenerative disorder with manifestations ranging from typical Parkinson disease to dementia with Lewy bodies. Clinical features include parkinsonian symptoms (resting tremor, rigidity, postural instability and bradykinesia), dementia, diffuse Lewy body pathology, autonomic dysfunction, hallucinations and paranoia.<ref>PMID:12408865</ref> <ref>PMID:9774100</ref> <ref>PMID:12705903</ref> <ref>PMID:16450370</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/UCHL1_HUMAN UCHL1_HUMAN] Ubiquitin-protein hydrolase involved both in the processing of ubiquitin precursors and of ubiquitinated proteins. This enzyme is a thiol protease that recognizes and hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin. Also binds to free monoubiquitin and may prevent its degradation in lysosomes. The homodimer may have ATP-independent ubiquitin ligase activity.<ref>PMID:9790970</ref> <ref>PMID:12408865</ref> <ref>PMID:18411255</ref>
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/et/2etl_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</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=2etl ConSurf].
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<div style="clear:both"></div>
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'''Crystal Structure of Ubiquitin Carboxy-terminal Hydrolase L1 (UCH-L1)'''
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==See Also==
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*[[Thioesterase 3D structures|Thioesterase 3D structures]]
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== References ==
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==Overview==
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<references/>
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The ubiquitin C-terminal hydrolase UCH-L1 (PGP9.5) comprises &gt;1% of total brain protein but is almost absent from other tissues [Wilkinson, K. D., et al. (1989) Science 246, 670-673]. Mutations in the UCH-L1 gene have been reported to be linked to susceptibility to and protection from Parkinson's disease [Leroy, E., et al. (1998) Nature 395, 451-452; Maraganore, D. M., et al. (1999) Neurology 53, 1858-1860]. Abnormal overexpression of UCH-L1 has been shown to correlate with several forms of cancer [Hibi, K., et al. (1998) Cancer Res. 58, 5690-5694]. Because the amino acid sequence of UCH-L1 is similar to that of other ubiquitin C-terminal hydrolases, including the ubiquitously expressed UCH-L3, which appear to be unconnected to neurodegenerative disease, the structure of UCH-L1 and the effects of disease associated mutations on the structure and function are of considerable importance. We have determined the three-dimensional structure of human UCH-L1 at 2.4-A resolution by x-ray crystallography. The overall fold resembles that of other ubiquitin hydrolases, including UCH-L3, but there are a number of significant differences. In particular, the geometry of the catalytic residues in the active site of UCH-L1 is distorted in such a way that the hydrolytic activity would appear to be impossible without substrate induced conformational rearrangements.
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__TOC__
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</StructureSection>
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==Disease==
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Known disease associated with this structure: Parkinson disease, familial OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=191342 191342]]
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==About this Structure==
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2ETL is a [[Single protein]] structure of 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=2ETL OCA].
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==Reference==
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Structural basis for conformational plasticity of the Parkinson's disease-associated ubiquitin hydrolase UCH-L1., Das C, Hoang QQ, Kreinbring CA, Luchansky SJ, Meray RK, Ray SS, Lansbury PT, Ringe D, Petsko GA, Proc Natl Acad Sci U S A. 2006 Mar 21;103(12):4675-80. Epub 2006 Mar 13. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/16537382 16537382]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Das, C.]]
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[[Category: Das C]]
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[[Category: Hoang, Q Q.]]
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[[Category: Hoang QQ]]
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[[Category: Kreinbring, C A.]]
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[[Category: Kreinbring CA]]
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[[Category: Lansbury, P T.]]
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[[Category: Lansbury PT]]
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[[Category: Luchansky, S J.]]
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[[Category: Luchansky SJ]]
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[[Category: Meray, R K.]]
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[[Category: Meray RK]]
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[[Category: Petsko, G A.]]
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[[Category: Petsko GA]]
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[[Category: Ray, S S.]]
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[[Category: Ray SS]]
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[[Category: Ringe, D.]]
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[[Category: Ringe D]]
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[[Category: Deubiquitinating thiol hydrolase]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun May 4 03:06:08 2008''
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Current revision

Crystal Structure of Ubiquitin Carboxy-terminal Hydrolase L1 (UCH-L1)

PDB ID 2etl

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