Ataxin-3 is a member of the polyglutamine family of proteins, which are associated with at
least nine different neurodegenerative diseases. In the disease state, expansion of the …

The polyglutamine diseases are a family of nine proteins where intracellular protein
misfolding and amyloid-like fibril formation are intrinsically coupled to disease. Previously …

Polyglutamine proteins that cause neurodegenerative disease are known to form
proteinaceous aggregates, such as nuclear inclusions, in the neurons of affected patients …

The misfolding and formation of fibrillar‐like aggregates by polyglutamine proteins is
believed to be a key factor in the development of the neurodegenerative polyglutamine …

A family of neurodegenerative diseases is associated with anomalous expansion of a
polyglutamine tract in the coding region of the corresponding proteins. The current working …

Machado-Joseph's disease is caused by a CAG trinucleotide repeat expansion that is
translated into an abnormally long polyglutamine tract in the protein ataxin-3. Except for the …

Anomalous expansion of a polyglutamine (polyQ) tract in the protein ataxin‐3 causes
spinocerebellar ataxia type 3, an autosomal dominant neurodegenerative disease. Very little …

Protein context clearly influences neurotoxicity in polyglutamine diseases, but the
contribution of alternative splicing to this phenomenon has rarely been investigated. Ataxin …

The protein ataxin-3 contains a polyglutamine region; increasing the number of glutamines
beyond 55 in this region gives rise to the neurodegenerative disease spinocerebellar ataxia …

The expansion of a trinucleotide (CAG) repeat, translated into a polyglutamine expanded
sequence in the protein encoded by the MJD gene, was identified over 20 years ago as the …