UCH-L1 is a member of the family of de-ubiquitinating enzymes responsible for mediating monomeric subunits from poly-ubiquitin chains (177). Mutations in UCH-L1 result in impaired clearance of ubiquitin and ubiquitinated proteins, therefore leading to elevated cell toxicity, protein accumulation, and cell death. At present, transgenic mice with altered UCH-L1 expression have been examined in the context of spermatogenesis and not extensively on alterations in basal ganglia function (178,179). Some studies have reported increased synuclein accumulation and neu-ropathology in UCH-L1 transgenic mice supporting interactions between synuclein and the UPS (180).
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