Abstract The 2004 Nobel Prize in chemistry for the discovery of protein ubiquitination has led
to the recognition of cellular proteolysis as a central area of research in biology. Eukaryotic …

Tandem repeat diseases include the neurodegenerative disorders known as polyglutamine
(polyQ) diseases, caused by CAG repeat expansions in the coding regions of the respective …

Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is
the most common inherited spinocerebellar ataxia and one of many polyglutamine …

Polyglutamine diseases are a major cause of neurodegeneration worldwide. Recent studies
highlight the importance of protein quality control mechanisms in regulating polyglutamine …

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 …

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 …

Selective protein degradation by the 26S proteasome requires the covalent attachment of
several ubiquitin molecules in the form of a multiubiquitin chain. Ubiquitylation usually …

The Hsp90/Hsp70-based chaperone machinery plays a well-established role in signaling
protein function, trafficking and turnover. A number of recent observations also support the …

Two central issues in polyglutamine-induced neurodegeneration are the influence of the
normal function of the disease protein and modulation by protein quality control pathways …

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 …