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Huntington's disease is one of nine known neurodegenerative disorders caused by an expanded polyglutamine (poly(Q)) tract in the disease protein. These diseases are associated with intraneuronal protein aggregates. Heat-inducible chaperones like HSP70 and HSP27 suppress poly(Q) aggregation and/or toxicity/cell death. Heat shock transcription factors,(More)
OBJECTIVE Nitrates are used widely in clinical practice. However, the mechanism underlying the bioactivation of nitrates to release NO remains unclear. Recent animal data suggest that mitochondrial aldehyde dehydrogenase (ALDH2) plays a central role in nitrate bioactivation, but its role in humans is not known. We investigated the role of ALDH2 in the(More)
T he formation of intracellular amyloid-like inclusions by mutant proteins is a feature of two groups of codon reiteration diseases, for which there are currently no treatments. The first group that was described includes the nine known neurodegenerative conditions caused by polyglutamine (polyQ) repeat expansions resulting from CAG trinucleotide repeat(More)
Department of Biochemistry, 2.205 Stopford Building, University of Manchester, Oxford Rd, Manchester M13 9PT, UK North West Lung Research Centre, South Manchester University Hospital, Wythenshaw, Southmoor Rd, Manchester M23 9LT, UK Department of Biomolecular Sciences, UMIST, PO Box 88, Sackville St, Manchester M60 1QD, UK Rhône-Poulenc Rorer Limited,(More)
Autosomal dominant oculopharyngeal muscular dystrophy (OPMD) is characterized pathologically by intranuclear inclusions in skeletal muscles and is caused by the expansion of a 10-alanine stretch to 12-17 alanines in the intranuclear poly(A)-binding protein 2 (PABP2). Whereas PABP2 is a major component of the inclusions in OPMD, the pathogenic mechanisms(More)
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