Polyglutamine Diseases: Where does Toxicity Come from? What is Toxicity? Where are We Going?

Phase 2 trial of leuprorelin in patients with spinal and bulbar muscular atrophy

(2009) Haruhiko Banno et al.   ANNALS OF NEUROLOGY

The promises and pitfalls of RNA-interference-based therapeutics

(2009) Daniela Castanotto et al.   NATURE

Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs

(2009) Jiaxin Hu et al.   NATURE BIOTECHNOLOGY

Rhes, a Striatal Specific Protein, Mediates Mutant-Huntingtin Cytotoxicity

(2009) S. Subramaniam et al.   SCIENCE

Polyglutamine Expansion Reduces the Association of TATA-binding Protein with DNA and Induces DNA Binding-independent Neurotoxicity

(2008) Meyer J. Friedman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Pathogenic Mechanisms of a Polyglutamine-mediated Neurodegenerative Disease, Spinocerebellar Ataxia Type 1

(2008) Huda Y. Zoghbi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1

(2008) Janghoo Lim et al.   NATURE