Article
Huntingtin fragmentation and increased caspase 3, 8 and 9 activities in lymphoblasts with heterozygous and homozygous Huntington's disease mutation.
Mechanisms of ageing and development - 1 Feb 2006
Maglione Vittorio, Cannella Milena, Gradini Roberto, Cislaghi Giuliana, Squitieri Ferdinando
Abstract excerpt
Huntington's disease (HD) is caused by mutated huntingtin (htt), a toxic protein ubiquitously expressed in nervous and non-nervous system tissues. Fragmentation of htt by caspases and further accumulation in cells of protein aggregates contribute to cell dysfunction and death. In the attempt to elucidate whether this mechanism depends on patients' genotype, we analysed the pattern of htt fragmentation, the...
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