The most frequent subtype of autosomal dominant inherited spinocerebellar ataxias is
caused by CAG repeat expansions of more than 55 units in the ataxin-3 gene. The clinical …

Accumulation of misfolded proteins in proteinaceous inclusions is a prominent pathological
feature common to many agerelated neurodegenerative diseases, including Parkinson's …

Ubiquitin chain complexity in cells is likely regulated by a diverse set of deubiquitinating
enzymes (DUBs) with distinct ubiquitin chain preferences. Here we show that the …

Spinocerebellar ataxia type 3 (SCA3), also known as Machado–Joseph disease (MJD), is
an autosomal-dominant neurodegenerative disorder caused by a polyglutamine expansion …

Elimination of misfolded proteins from the endoplasmic reticulum (ER) by ER-associated
degradation involves substrate retrotranslocation from the ER lumen into the cytosol for …

Systematic pathoanatomical analysis of spinocerebellar ataxia type 3 brains helps to
understand the structural basis of this neurodegenerative disease and offers explanations …

In the present study, we prepared a SCA3 animal model by generating transgenic mice
expressing polyglutamine-expanded ataxin-3-Q79. Ataxin-3-Q79 was expressed in brain …

Ubiquitin‐specific proteases (UBPs) are a highly conserved family of proteins in eukaryotes,
and play critical roles in protein de‐ubiquitination. Here we report a systematic genetic and …

Background Reversible modification of proteins through the attachment of ubiquitin or
ubiquitin-like modifiers is an essential post-translational regulatory mechanism in …

Machado-Joseph disease (MJD) is a fatal, dominant neurodegenerative disorder. MJD
results from polyglutamine repeat expansion in the MJD-1 gene, conferring a toxic gain of …