Mechanisms of deubiquitinase specificity and regulation

TET Mevissen, D Komander - Annual review of biochemistry, 2017 - annualreviews.org
Protein ubiquitination is one of the most powerful posttranslational modifications of proteins,
as it regulates a plethora of cellular processes in distinct manners. Simple …

Deubiquitylases from genes to organism

MJ Clague, I Barsukov, JM Coulson… - Physiological …, 2013 - journals.physiology.org
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 …

Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7

U Rüb, L Schöls, H Paulson, G Auburger… - Progress in …, 2013 - Elsevier
The spinocerebellar ataxias type 1 (SCA1), 2 (SCA2), 3 (SCA3), 6 (SCA6) and 7 (SCA7) are
genetically defined autosomal dominantly inherited progressive cerebellar ataxias (ADCAs) …

[HTML][HTML] Pathogenesis of SCA3 and implications for other polyglutamine diseases

HS McLoughlin, LR Moore, HL Paulson - Neurobiology of disease, 2020 - Elsevier
Tandem repeat diseases include the neurodegenerative disorders known as polyglutamine
(polyQ) diseases, caused by CAG repeat expansions in the coding regions of the respective …

The predator becomes the prey: regulating the ubiquitin system by ubiquitylation and degradation

AM Weissman, N Shabek, A Ciechanover - Nature reviews Molecular …, 2011 - nature.com
Ubiquitylation (also known as ubiquitination) regulates essentially all of the intracellular
processes in eukaryotes through highly specific modification of numerous cellular proteins …

Toward understanding Machado–Joseph disease

M do Carmo Costa, HL Paulson - Progress in neurobiology, 2012 - Elsevier
Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is
the most common inherited spinocerebellar ataxia and one of many polyglutamine …

Post-translational modifications of deubiquitinating enzymes: expanding the ubiquitin code

Y Wang, F Wang - Frontiers in pharmacology, 2021 - frontiersin.org
Post-translational modifications such as ubiquitination play important regulatory roles in
several biological processes in eukaryotes. This process could be reversed by …

Post-translational modifications: regulators of neurodegenerative proteinopathies

R Gupta, M Sahu, D Srivastava, S Tiwari… - Ageing Research …, 2021 - Elsevier
One of the hallmark features in the neurodegenerative disorders (NDDs) is the accumulation
of aggregated and/or non-functional protein in the cellular milieu. Post-translational …

Machado–Joseph disease/spinocerebellar ataxia type 3: lessons from disease pathogenesis and clues into therapy

CA Matos, LP de Almeida… - Journal of …, 2019 - Wiley Online Library
Abstract Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3
(SCA 3), is an incurable disorder, widely regarded as the most common form of …

Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP

KM Scaglione, E Zavodszky, SV Todi, S Patury, P Xu… - Molecular cell, 2011 - cell.com
The mechanisms by which ubiquitin ligases are regulated remain poorly understood. Here
we describe a series of molecular events that coordinately regulate CHIP, a neuroprotective …