Polyglutamine diseases: looking beyond the neurodegenerative universe

Beyond the Qs in the polyglutamine diseases.

Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle.

The polyglutamine disorders are a class of nine neuro-degenerative disorders that are inherited gain-of-function diseases caused by expansion of a translated CAG repeat. Even though the disease-causing proteins are widely expressed, specific collections of neurons are more susceptible in each disease, resulting in characteristic patterns of pathology and clinical symptoms. One hypothesis poses ...

Canadian Association of Neurosciences Review: polyglutamine expansion neurodegenerative diseases.

Since the early 1990s, DNA triplet repeat expansions have been found to be the cause in an ever increasing number of genetic neurologic diseases. A subset of this large family of genetic diseases has the expansion of a CAG DNA triplet in the open reading frame of a coding exon. The result of this DNA expansion is the expression of expanded glutamine amino acid repeat tracts in the affected prot...

Molecular Origin of Polyglutamine Aggregation in Neurodegenerative Diseases

Expansion of polyglutamine (polyQ) tracts in proteins results in protein aggregation and is associated with cell death in at least nine neurodegenerative diseases. Disease age of onset is correlated with the polyQ insert length above a critical value of 35-40 glutamines. The aggregation kinetics of isolated polyQ peptides in vitro also shows a similar critical-length dependence. While recent ex...

Frontier in Neurology Research Beyond Neurodegenerative Diseases

Editorial As a guest editor for this special issue of Neurology Diseases in the Chinese Medical Journal (English Edition), I am pleased to announce a completion of 19 articles (13 research articles, 4 review papers and 2 case reports) which cover a spectrum of research frontier in neurology diseases for publication in this issue. These articles provide an update on the state of clinical and bas...