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 (Comment) | Organism |
---|---|
expressed in COS-7 cells | Homo sapiens |
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 | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
cytoplasm | - |
Homo sapiens | 5737 | - |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
25000 | - |
immunoblot analysis | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | P09936 | - |
- |
Mus musculus | - |
- |
- |
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 | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
ubiquitin-7-amido-4-methylcoumarin + H2O | - |
Homo sapiens | ubiquitin + 7-amino-4-methylcoumarin | - |
? |
Synonyms | Comment | Organism |
---|---|---|
ubiquitin C-terminal hydrolase | - |
Mus musculus |
ubiquitin C-terminal hydrolase | - |
Homo sapiens |
UCH-L1 | - |
Mus musculus |
UCH-L1 | - |
Homo sapiens |