The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution
NP Dantuma, LC Bott - Frontiers in molecular neuroscience, 2014 - frontiersin.org
The ubiquitin-proteasome system (UPS) has been implicated in neurodegenerative
diseases based on the presence of deposits consisting of ubiquitylated proteins in affected …
diseases based on the presence of deposits consisting of ubiquitylated proteins in affected …
[HTML][HTML] Regulation of proteolysis by human deubiquitinating enzymes
ZM Eletr, KD Wilkinson - Biochimica et Biophysica Acta (BBA)-Molecular …, 2014 - Elsevier
The post-translational attachment of one or several ubiquitin molecules to a protein
generates a variety of targeting signals that are used in many different ways in the cell …
generates a variety of targeting signals that are used in many different ways in the cell …
Ataxin-3 protein and RNA toxicity in spinocerebellar ataxia type 3: current insights and emerging therapeutic strategies
MM Evers, LJA Toonen, WMC van Roon-Mom - Molecular neurobiology, 2014 - Springer
Ataxin-3 is a ubiquitously expressed deubiqutinating enzyme with important functions in the
proteasomal protein degradation pathway and regulation of transcription. The C-terminus of …
proteasomal protein degradation pathway and regulation of transcription. The C-terminus of …
An optimal ubiquitin-proteasome pathway in the nervous system: the role of deubiquitinating enzymes
The Ubiquitin-Proteasome Pathway (UPP), which is critical for normal function in the
nervous system and is implicated in various neurological diseases, requires the small …
nervous system and is implicated in various neurological diseases, requires the small …
The E3 ligase CHIP: insights into its structure and regulation
The carboxy‐terminus of Hsc70 interacting protein (CHIP) is a cochaperone E3 ligase
containing three tandem repeats of tetratricopeptide (TPR) motifs and a C‐terminal U‐box …
containing three tandem repeats of tetratricopeptide (TPR) motifs and a C‐terminal U‐box …
RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of Machado-Joseph disease
Machado-Joseph disease or Spinocerebellar ataxia type 3 is a progressive fatal
neurodegenerative disorder caused by the polyglutamine-expanded protein ataxin-3 …
neurodegenerative disorder caused by the polyglutamine-expanded protein ataxin-3 …
Emerging evidence of coding mutations in the ubiquitin–proteasome system associated with cerebellar ataxias
SM Ronnebaum, C Patterson, JC Schisler - Human Genome Variation, 2014 - nature.com
Cerebellar ataxia (CA) is a disorder associated with impairments in balance, coordination,
and gait caused by degeneration of the cerebellum. The mutations associated with CA affect …
and gait caused by degeneration of the cerebellum. The mutations associated with CA affect …
p62/sequestosome 1 regulates aggresome formation of pathogenic ataxin-3 with expanded polyglutamine
L Zhou, H Wang, D Chen, F Gao, Z Ying… - International journal of …, 2014 - mdpi.com
The cellular protein quality control system in association with aggresome formation
contributes to protecting cells against aggregation-prone protein-induced toxicity …
contributes to protecting cells against aggregation-prone protein-induced toxicity …
DNA damage emergency: cellular garbage disposal to the rescue?
HR Stone, JR Morris - Oncogene, 2014 - nature.com
The proteasome is a cellular machine found in the cytosol, nucleus and on chromatin that
performs much of the proteolysis in eukaryotic cells. Recent reports show it is enriched at …
performs much of the proteolysis in eukaryotic cells. Recent reports show it is enriched at …
Interactions of ataxin-3 with its molecular partners in the protein machinery that sorts protein aggregates to the aggresome
M Bonanomi, S Mazzucchelli, A D'Urzo… - The international journal …, 2014 - Elsevier
Abstract Ataxin-3 (AT3) is the protein that triggers the inherited neurodegenerative disorder
spinocerebellar ataxia type 3 when its polyglutamine (polyQ) stretch close to the C-terminus …
spinocerebellar ataxia type 3 when its polyglutamine (polyQ) stretch close to the C-terminus …