Spinocerebellar ataxia type 3 is a neurodegenerative disorder caused by the expansion of
the polyglutamine repeat region within the ataxin-3 protein. The mutant protein forms …

Polyglutamine (polyQ) diseases are associated with a CAG/polyQ expansion mutation in
unrelated proteins. Upon elongation of the glutamine tract, disease proteins aggregate …

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 …

Removal of ubiquitin from modified proteins is an important process to regulate the ubiquitin
system. Roughly 100 dedicated enzymes for this purpose, the deubiquitinases, exist in …

Deubiquitinating enzymes (DUbs) play important roles in many ubiquitin-dependent
pathways, yet how DUbs themselves are regulated is not well understood. Here, we provide …

Abstract Spinocerebellar ataxia type 3 (SCA3)/Machado Joseph disease results from
expansion of the polyglutamine domain in ataxin-3 (Atx3). Atx3 is a transcriptional co …

Protein context clearly influences neurotoxicity in polyglutamine diseases, but the
contribution of alternative splicing to this phenomenon has rarely been investigated. Ataxin …

Ataxin-3 (ATXN3) is a widely expressed protein that binds to ubiquitylated proteins, has
deubiquitylating activity in vitro and is thought to modulate substrate degradation through the …

Ataxin-3, the disease protein in the neurodegenerative disorder Spinocerebellar Ataxia Type
3 or Machado Joseph disease, is a cysteine protease implicated in the ubiquitin proteasome …

The ubiquitin-interacting motif (UIM) is a short peptide with dual function of binding ubiquitin
(Ub) and promoting ubiquitination. We elucidated the structures and dynamics of the tandem …