Over the past decade, remarkable advances have been witnessed in the development of
small-molecule probes. These molecular tools have been widely applied for interrogating …

Ubiquitylation is a major posttranslational modification that controls most complex aspects of
cell physiology. It is reversed through the action of a large family of deubiquitylating …

Ubiquitin C-terminal hydrolase L1 (UCH-L1) is an extremely abundant protein in the brain
where, remarkably, it is estimated to make up 1–5% of total neuronal protein. Although it …

Autosomal‐dominant spinocerebellar ataxias, autosomal‐recessive spinocerebellar ataxias,
and hereditary spastic paraplegias have traditionally been designated in separate …

Hereditary spastic paraplegias (HSP) are a heterogeneous group of motor
neurodegenerative disorders that have the core clinical presentation of pyramidal syndrome …

Ubiquitination regulates protein homeostasis and is tightly controlled by deubiquitinases
(DUBs). Loss of the DUB UCHL1 leads to neurodegeneration, and its dysregulation …

There is currently no accepted classification of autosomal recessive cerebellar ataxias, a
group of disorders characterized by important genetic heterogeneity and complex …

UCHL1 is a multifunctional protein expressed at high concentrations in neurons in the brain
and spinal cord. UCHL1 plays important roles in regulating the level of cellular free ubiquitin …

The human genome encodes a vast repertoire of protein non-coding RNAs (ncRNA), some
specific to the brain. MicroRNAs, which interfere with the translation of target mRNAs, are of …

The role of ubiquitin carboxyl-terminal hydrolase L1 (UCHL1; also called PARK5) in the
pathogenesis of Parkinson's disease (PD) has been controversial. Here, we find that the loss …