[43] Detection of polyglutamine aggregation in mouse models
SW Davies, K Sathasivam, C Hobbs, P Doherty… - Methods in …, 1999 - Elsevier
Publisher Summary The first steps in the molecular pathogenesis of HD are beginning to be
unraveled. The huntingtin protein is subjected to an initial cleavage step that releases an N-
terminal fragment. The precise nature of this fragment and the cleavage mechanism are
unknown, although caspase 3 has been proposed to fill this role. The N-terminal fragment
can move into the nucleus, but once again, the mechanism by which this occurs is unknown.
Nuclear inclusions have been described in juvenile onset HD with a frequency of 38–50% in …
unraveled. The huntingtin protein is subjected to an initial cleavage step that releases an N-
terminal fragment. The precise nature of this fragment and the cleavage mechanism are
unknown, although caspase 3 has been proposed to fill this role. The N-terminal fragment
can move into the nucleus, but once again, the mechanism by which this occurs is unknown.
Nuclear inclusions have been described in juvenile onset HD with a frequency of 38–50% in …