Background: Spinocerebellar ataxia type 10 (SCA10) is an autosomal dominant disorder
characterized by cerebellar ataxia and seizures. SCA10 is caused by an expansion of an …

Transcriptional dysregulation has been described in spinocerebellar ataxia type 3/Machado-
Joseph disease (SCA3/MJD), an autosomal dominant ataxia caused by a polyglutamine …

The RNA-binding protein Ataxin-2 binds to and stabilizes a number of mRNA sequences,
including that of the transactive response DNA-binding protein of 43 kDa (TDP-43). Ataxin-2 …

Spinocerebellar ataxia type 3 (SCA3) or Machado–Joseph disease (MJD) belongs to a
group of autosomal dominant neurodegenerative diseases, which are caused by the …

Abstract Spinocerebellar ataxia type 7 (SCA7) is an autosomal dominant polyglutamine
disorder presenting with progressive cerebellar ataxia and blindness. The molecular …

Summary Spinocerebellar ataxia type 1 (SCA1) is a paradigmatic neurodegenerative
disease in that it is caused by a mutation in a broadly expressed protein, ATXN1; however …

The polyglutamine-containing neurodegenerative protein ataxin 3 (AT3) has
deubiquitylating activity and binds ubiquitin chains with a preference for chains of four or …

Spinocerebellar ataxia 2 (SCA2) is a neurodegenerative disorder characterized by
progressive ataxia. SCA2 results from a poly (Q)(polyglutamine) expansion in the cytosolic …

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 …

Abstract Spinocerebellar ataxia type 2 (SCA2) and amyotrophic lateral sclerosis (ALS) are
neurodegenerative disorders, caused or modified by an unstable CAG-repeat expansion in …