3kvf: Difference between revisions
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{{STRUCTURE_3kvf| PDB=3kvf | SCENE= }} | {{STRUCTURE_3kvf| PDB=3kvf | SCENE= }} | ||
===Crystal structure of the I93M mutant of ubiquitin carboxy terminal hydrolase L1 bound to ubiquitin vinylmethylester=== | |||
{{ABSTRACT_PUBMED_20439756}} | |||
=== | ==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> | |||
==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> | |||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
<ref group="xtra">PMID:020439756</ref><references group="xtra"/> | <ref group="xtra">PMID:020439756</ref><references group="xtra"/><references/> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Das, C.]] | [[Category: Das, C.]] | ||