Deubiquitinases in cancer: new functions and therapeutic options

JM Fraile, V Quesada, D Rodríguez, JMP Freije… - Oncogene, 2012 - nature.com
Deubiquitinases (DUBs) have fundamental roles in the ubiquitin system through their ability
to specifically deconjugate ubiquitin from targeted proteins. The human genome encodes at …

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 …

Machado-Joseph Disease: from first descriptions to new perspectives

C Bettencourt, M Lima - Orphanet journal of rare diseases, 2011 - Springer
Abstract Machado-Joseph Disease (MJD), also known as spinocerebellar ataxia type 3
(SCA3), represents the most common form of SCA worldwide. MJD is an autosomal …

Genome-Wide Screen for Modifiers of Ataxin-3 Neurodegeneration in Drosophila

J Bilen, NM Bonini - PLoS genetics, 2007 - journals.plos.org
Spinocerebellar ataxia type-3 (SCA3) is among the most common dominantly inherited
ataxias, and is one of nine devastating human neurodegenerative diseases caused by the …

Mechanisms, biology and inhibitors of deubiquitinating enzymes

KR Love, A Catic, C Schlieker, HL Ploegh - Nature chemical biology, 2007 - nature.com
The addition of ubiquitin (Ub) and ubiquitin-like (Ubl) modifiers to proteins serves to
modulate function and is a key step in protein degradation, epigenetic modification and …

Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination

I Schmitt, M Linden, H Khazneh, BO Evert… - Biochemical and …, 2007 - Elsevier
Spinocerebellar ataxia type 3 is a neurodegenerative disease caused by expansion of a
polyglutamine domain in the protein ataxin-3 (ATXN3). Physiological functions of ATXN3 …

Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado–Joseph …

AT Simoes, N Gonçalves, A Koeppen, N Deglon… - Brain, 2012 - academic.oup.com
Machado–Joseph disease is the most frequently found dominantly-inherited cerebellar
ataxia. Over-repetition of a CAG trinucleotide in the MJD1 gene translates into a …

Mechanisms orchestrating the enzymatic activity and cellular functions of deubiquitinases

B Estavoyer, C Messmer, M Echbicheb… - Journal of Biological …, 2022 - ASBMB
Deubiquitinases (DUBs) are required for the reverse reaction of ubiquitination and act as
major regulators of ubiquitin signaling processes. Emerging evidence suggests that these …

Silencing ataxin-3 mitigates degeneration in a rat model of Machado–Joseph disease: no role for wild-type ataxin-3?

S Alves, I Nascimento-Ferreira, N Dufour… - Human molecular …, 2010 - academic.oup.com
Machado–Joseph disease or spinocerebellar ataxia type 3 (MJD/SCA3) is a fatal, autosomal
dominant disorder caused by a cytosine-adenine-guanine expansion in the coding region of …

From pathways to targets: understanding the mechanisms behind polyglutamine disease

JJ Weber, AS Sowa, T Binder… - BioMed research …, 2014 - Wiley Online Library
The history of polyglutamine diseases dates back approximately 20 years to the discovery of
a polyglutamine repeat in the androgen receptor of SBMA followed by the identification of …