4jkj

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== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[4jkj]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4JKJ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4JKJ FirstGlance]. <br>
<table><tr><td colspan='2'>[[4jkj]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4JKJ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4JKJ FirstGlance]. <br>
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</td></tr><tr><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene><br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
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<tr><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2etl|2etl]]</td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2etl|2etl]]</td></tr>
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<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Ubiquitinyl_hydrolase_1 Ubiquitinyl hydrolase 1], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.4.19.12 3.4.19.12] </span></td></tr>
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<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Ubiquitinyl_hydrolase_1 Ubiquitinyl hydrolase 1], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.4.19.12 3.4.19.12] </span></td></tr>
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<tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4jkj FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4jkj OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4jkj RCSB], [http://www.ebi.ac.uk/pdbsum/4jkj PDBsum]</span></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4jkj FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4jkj OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4jkj RCSB], [http://www.ebi.ac.uk/pdbsum/4jkj PDBsum]</span></td></tr>
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<table>
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</table>
== Disease ==
== Disease ==
[[http://www.uniprot.org/uniprot/UCHL1_HUMAN UCHL1_HUMAN]] Defects in UCHL1 are the cause of Parkinson disease type 5 (PARK5) [MIM:[http://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>
[[http://www.uniprot.org/uniprot/UCHL1_HUMAN UCHL1_HUMAN]] Defects in UCHL1 are the cause of Parkinson disease type 5 (PARK5) [MIM:[http://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>
== Function ==
== Function ==
[[http://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>
[[http://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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==See Also==
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*[[Thioesterase|Thioesterase]]
== References ==
== References ==
<references/>
<references/>
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</StructureSection>
</StructureSection>
[[Category: Ubiquitinyl hydrolase 1]]
[[Category: Ubiquitinyl hydrolase 1]]
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[[Category: Das, C.]]
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[[Category: Das, C]]
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[[Category: Davies, C W.]]
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[[Category: Davies, C W]]
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[[Category: Petsko, G A.]]
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[[Category: Petsko, G A]]
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[[Category: Ringe, D.]]
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[[Category: Ringe, D]]
[[Category: Hydrolase]]
[[Category: Hydrolase]]
[[Category: Ubiquitin hydrolase]]
[[Category: Ubiquitin hydrolase]]

Revision as of 13:14, 4 January 2015

Crystal Structure of the S18Y Variant of Ubiquitin Carboxy-terminal Hydrolase L1

4jkj, resolution 2.15Å

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