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Literature summary for 3.4.19.12 extracted from

  • Barrachina, M.; Castano, E.; Dalfo, E.; Maes, T.; Buesa, C.; Ferrer, I.
    Reduced ubiquitin C-terminal hydrolase-1 expression levels in dementia with Lewy bodies (2006), Neurobiol. Dis., 22, 265-273.
    View publication on PubMed

Application

Application Comment Organism
medicine UCHL-1 expression is a contributory factor in the abnormal protein aggregation in dementia with Lewy bodies. Putative therapeutic target in the treatment of dementia with Lewy bodies Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
cytoplasm UCHL-1 in the cytoplasm of neurons in the cerebral cortex and medulla oblongata in control and diseased brains Homo sapiens 5737
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Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens the enzyme is involved in generation of free monomeric ubiquitin, enzyme deficiency leads is involved in accumulation of ubiquitin and alpha-synuclein leading to aggregation in proteins conforming Lewy bodies and Lewy neurites leading to Parkinson's disease and dementia with Lewy bodies, the enzyme is down-regulated in Alzheimer's disease and in Parkinson's disease, comparison of enzyme expression to general protein synthesis and proteasome activity in brain ?
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?

Organism

Organism UniProt Comment Textmining
Homo sapiens
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-
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Source Tissue

Source Tissue Comment Organism Textmining
brain
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Homo sapiens
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brain frontal cortex Homo sapiens
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cerebral cortex
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Homo sapiens
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cerebral cortex down-regulation of UCHL-1 mRNA and protein in dementia with Lewy bodies, either in pure forms not associated with Alzheimer disease, and in common forms, with accompanying Alzheimer disease changes, but not in Parkinson disease. UCHL-3 expression reduced in Parkinson disease and dementia with Lewy bodies Homo sapiens
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medulla oblongata
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Homo sapiens
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medulla oblongata UCHL-1 mRNA and protein expressions reduced in Parkinson disease Homo sapiens
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neuron
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Homo sapiens
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substantia nigra UCHL-1 protein reduced in cases with Lewy body pathology Homo sapiens
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information the enzyme is involved in generation of free monomeric ubiquitin, enzyme deficiency leads is involved in accumulation of ubiquitin and alpha-synuclein leading to aggregation in proteins conforming Lewy bodies and Lewy neurites leading to Parkinson's disease and dementia with Lewy bodies, the enzyme is down-regulated in Alzheimer's disease and in Parkinson's disease, comparison of enzyme expression to general protein synthesis and proteasome activity in brain Homo sapiens ?
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?

Synonyms

Synonyms Comment Organism
ubiquitin C-terminal hydrolase-1
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Homo sapiens
UCHL-1
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Homo sapiens
UCHL-3
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Homo sapiens