IndraLab

Statements

UCHL1 inhibits HMBS. 6 / 6
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"Inhibition of UCH-L1 hydrolase function in MN reduced UPS impairment and ameliorated proteinuria."
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"In support of this hypothesis, genetic mutations of Parkin and UCHL-1, which are responsible for inherited PD, decrease the enzymatic activity of the UPS (Kitada et al., 1998; Leroy et al., 1998)."
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"Decreased UCHL1 protein levels cause a reduction in the amount of monomeric ubiquitin in neurons and induce damage of the UPS [27] ."
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"Moreover, UCHL-1 mutation (I93M) reported in autosomal dominant PD reduces catalytic UPS activity in vitro (Nishikawa et al., 2003), and is associated with parkinsonism, cognitive deficits and cortica[MISSING/INVALID CREDENTIALS: limited to 200 char for Elsevier]"
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"Pharmacological inhibition of UCHL1 in this model restores UPS activity, decreases poly-ubiquitinated proteins and ameliorates the overall glomerular pathology [21] ."
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"UCHL1 deletion is predicted to restore the UPS."
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