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Literature summary for 3.4.19.12 extracted from

  • Bifsha, P.; Landry, K.; Ashmarina, L.; Durand, S.; Seyrantepe, V.; Trudel, S.; Quiniou, C.; Chemtob, S.; Xu, Y.; Gravel, R.A.; Sladek, R.; Pshezhetsky, A.V.
    Altered gene expression in cells from patients with lysosomal storage disorders suggests impairment of the ubiquitin pathway (2007), Cell Death Differ., 14, 511-523.
    View publication on PubMed

Application

Application Comment Organism
medicine UCH-L1 deficiency and impairment of the ubiquitin-dependent protein degradation pathway contributes to increased cell death observed in many lysosomal storage disorders Mus musculus
medicine UCH-L1 deficiency and impairment of the ubiquitin-dependent protein degradation pathway contributes to increased cell death observed in many lysosomal storage disorders Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
expressed in COS-7 cells Homo sapiens

Inhibitors

Inhibitors Comment Organism Structure
epoxysuccinyl-leucylamido-(4-guanidino) butane reduces UCH-L1 mRNA, protein level and activity. Caspase-mediated apoptosis in epoxysuccinyl-leucylamido-(4-guanidino) butane-treated fibroblasts is reversed by transfection with a UCH-L1 plasmid Homo sapiens
siRNA inhibits the expression of UCH-L1 in normal control fibroblasts, followed by a remarkable increase of apoptosis both in the presence and in the absence of epoxysuccinyl-leucylamido-(4-guanidino) butane. UCH-L1 siRNA 14311 almost completely inhibits synthesis of UCH-L1 protein in about 70% of cells and reduces the UCH-L1 mRNA level more then 7fold Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
cytoplasm
-
Homo sapiens 5737
-

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
25000
-
immunoblot analysis Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens P09936
-
-
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain reduced levels of UCH-L1 mRNA (30%) and protein in a mouse model of Sandhoff disease as compared with their wild-type siblings Mus musculus
-
skin fibroblast level of UCH-L1 mRNA is significantly reduced in fibroblasts of patients affected with lysosomal storage disorders Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ubiquitin-7-amido-4-methylcoumarin + H2O
-
Homo sapiens ubiquitin + 7-amino-4-methylcoumarin
-
?

Synonyms

Synonyms Comment Organism
ubiquitin C-terminal hydrolase
-
Mus musculus
ubiquitin C-terminal hydrolase
-
Homo sapiens
UCH-L1
-
Mus musculus
UCH-L1
-
Homo sapiens