Introduction Six of the most frequent dominantly inherited spinocerebellar ataxias (SCAs)
worldwide–SCA1, SCA2, SCA3, SCA6, SCA7, and SCA17–are caused by an expansion of a …

Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant neurodegenerative disorder
that gives rise to motor incoordination and progressive functional disabilities. Although …

Nine polyglutamine (polyQ) proteins have already been identified that are considered to be
associated with the pathologies of neurodegenerative disorders called polyQ diseases, but …

We developed a method that utilizes fluorescent labeling of nuclear envelopes alongside
cytometry sorting for the selective isolation of Purkinje cell (PC) nuclei. Beginning with SUN1 …

Human neurodegenerative polyglutamine [poly (Q)] disorders, such as Huntington's disease
(HD) and spinocerebellar ataxias (SCA), are characterised by an abnormal expansion of …

Abstract Spinocerebellar ataxia type 7 (SCA7) is an inherited neurodegenerative disorder
caused by a CAG-polyglutamine repeat expansion. SCA7 patients display a striking loss of …

Spinocerebellar ataxias (SCAs) are a rare autosomal dominant neurodegenerative disorder.
To date, approximately 50 different subtypes of SCAs have been characterized. The …

Abstract Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease, is
a neurodegenerative disease caused by expanded polyglutamine repeats in exon 10 of the …

One of the characteristic areas of brainstem degeneration across multiple spinocerebellar
ataxias (SCAs) is the inferior olive (IO), a medullary nucleus that plays a key role in motor …

Abstract Spinocerebellar ataxia type 7 (SCA7) is a cerebellar and retinal neurodegenerative
disease caused by a CAG/polyglutamine (polyQ) expansion mutation in the ataxin-7 …