Article
Conformational dynamics and self-association of intrinsically disordered Huntingtin exon 1 in cells.
Physical chemistry chemical physics : PCCP - 3 May 2017
Büning Steffen, Sharma Abhishek, Vachharajani Shivang, Newcombe Estella, Ormsby Angelique, Gao Mimi, Gnutt David, Vöpel Tobias, Hatters Danny M, Ebbinghaus Simon
Abstract excerpt
Huntington's disease is caused by a CAG trinucleotide expansion mutation in the Huntingtin gene that leads to an artificially long polyglutamine sequence in the Huntingtin protein. A key feature of the disease is the intracellular aggregation of the Huntingtin exon 1 protein (Httex1) into micrometer sized inclusion bodies. The aggregation process of Httex1 has been extensively studied in vitro, however, the...
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