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 …

Spinocerebellar ataxia type 1 (SCA1) is an adult-onset neurodegenerative disease caused
by a polyglutamine expansion in the protein ATXN1, which is involved in transcriptional …

Abstract Background Machado–Joseph Disease (MJD), a form of dominantly inherited
ataxia belonging to the group of polyQ expansion neurodegenerative disorders, occurs …

The spinocerebellar ataxia type 7 (SCA7) gene product, Ataxin-7 (ATXN7), localizes to the
nucleus and has been shown to function as a component of the TATA-binding protein-free …

Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant neurodegenerative disease
caused by polyglutamine-expanded ataxin-3. Previously, we prepared a SCA3 animal …

Spinocerebellar ataxia type 1 (SCA1) is a lethal neurodegenerative disorder caused by
expansion of a polyglutamine tract in ATXN1. A prominent site of pathology in SCA1 is …

In this work we investigate subcellular localization and proteolytic cleavage of different forms
of ataxin-3 (AT-3), the protein responsible for spinocerebellar ataxia type 3. Normal (AT-3Q6 …

The nuclear presence of the expanded disease proteins is of critical importance for the
pathogeneses of polyglutamine diseases. Here we show that protein casein kinase 2 (CK2) …

Spinocerebellar ataxia type 1 is an autosomal dominant cerebellar ataxia associated with
the expansion of a polyglutamine tract within the ataxin-1 (ATXN1) protein. Recent studies …

Abstract Ataxin 1 (Atxn1) is a protein of unknown function associated with spinocerebellar
ataxia type 1 (SCA1), a neurodegenerative disease of late onset with variable degrees of …