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Literature summary for 3.4.19.12 extracted from

  • Gong, B.; Leznik, E.
    The role of ubiquitin C-terminal hydrolase L1 in neurodegenerative disorders (2007), Drug News Perspect., 20, 365-370.
    View publication on PubMed

Application

Application Comment Organism
medicine UCH-L1 is an important component of the ubiquitin-proteasome system and is linked to Parkinson´s, Huntington´s, Alzheimer´s disease and other neurodegenerative disorders. UCH-L1 is an attractive target for the development of new therapeutic approaches to Alzheimer´s disease Homo sapiens

Protein Variants

Protein Variants Comment Organism
I93M missense mutation in the UCH-L1 gene in a German family with a familial case of Parkinson´s disease, decreases hydrolytic activity Homo sapiens
S18Y reduces risk of Parkinson´s disease. The protective role reflects a restricted mechanism only applied in sporadic Parkinson´s disease patients. The mutant is less likely to form dimers. Mutation also present in patients with earlier/later onset of Huntington´s disease Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
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Source Tissue

Source Tissue Comment Organism Textmining
brain highly expressed. UCH-L1 expression and protein level are downregulated in the brain of patients with Alzheimer´s disease Homo sapiens
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lewy body present in patients with sporadic Parkinson´s disease Homo sapiens
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neurofibrillary tangle present in patients with Alzheimer´s disease Homo sapiens
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information UCH-L1 may directly destroy or neutralize oligomeric beta-amyloid peptide and may promote degradation of beta- and gamma secretase, or amyloid precursor protein Homo sapiens ?
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?
ubiquitin + H2O UCH-L1 removes and recycles ubiquitin molecules from degraded proteins Homo sapiens monoubiquitin
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?

Synonyms

Synonyms Comment Organism
PGP9.5.1
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Homo sapiens
ubiquitin C-terminal hydrolase L1
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Homo sapiens
UCH-L1
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Homo sapiens