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Literature summary for 3.4.19.12 extracted from

  • Radon, V.; Czesla, M.; Reichelt, J.; Fehlert, J.; Hammel, A.; Rosendahl, A.; Knop, J.H.; Wiech, T.; Wenzel, U.O.; Sachs, M.; Reinicke, A.T.; Stahl, R.A.K.; Meyer-Schwesinger, C.
    Ubiquitin C-terminal hydrolase L1 is required for regulated protein degradation through the ubiquitin proteasome system in kidney (2018), Kidney Int., 93, 110-127 .
    View publication on PubMed

Organism

Organism UniProt Comment Textmining
Mus musculus Q9R0P9
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Source Tissue

Source Tissue Comment Organism Textmining
kidney the enzyme is found in tubular and parietal cells of the kidney and is expressed de novo in injured podocytes. Constitutive UCH-L1 expression in tubulointerstitial and glomerular cells Mus musculus
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podocyte the enzyme is found in tubular and parietal cells of the kidney and is expressed de novo in injured podocytes Mus musculus
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Synonyms

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

General Information

General Information Comment Organism
malfunction UCH-L1-deficient mice develop proteinuria, without gross changes in glomerular morphology. Tubular cells, endothelial cells, and podocytes show signs of stress with an accumulation of oxidative modified and polyubiquitinated proteins. Mechanistically, abnormal protein accumulation results from an altered proteasome abundance leading to decreased proteasomal activity. UCH-L1-deficient mice exhibit an exacerbated course of disease with increased tubulointerstitial and glomerular damage, acute renal failure, and death, the latter most likely a result of general neurologic impairment Mus musculus
metabolism the enzyme is required for regulated protein degradation through the ubiquitin proteasome system in kidney Mus musculus
physiological function major deubiquitinating enzyme of the nervous system and associated with the development of neurodegenerative diseases Mus musculus
physiological function the enzyme is required for regulated protein degradation in the kidney by controlling proteasome abundance Mus musculus