Sentences with phrase «mutant huntingtin on»
«Because mitochondrial dysfunction has been proposed to play an important role in the pathogenesis of Huntington's disease,» said Qi, «we investigated the binding proteins of mutant huntingtin on mitochondria.»
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This abnormal binding causes more VCP accumulation on the mitochondria,» Nerve cells with VCP - mutant huntingtin interacting inside them became dysfunctional and self - destructed.
To further explore nuclear transport's role in Huntington's disease, Grima took lab - grown mouse neurons and used chemical switches to a) turn on both an additional healthy copy of the RanGAP1 gene and a mutant version of Huntingtin; b) just turn on the mutant Huntingtin; or c) just turn on a healthy version of Huntingtin.
Any effects were small compared with the desirable action on the mutant huntingtin gene.
When a person has too many CAGs in a row near the start of the huntingtin gene, a harmful «mutant» form of the protein is produced based on the instructions in the gene.
Given the adult onset and dysfunction and death of adult neurons characterizing HD, most studies have focused on the toxic effects elicited by mutant huntingtin in post-mitotic neurons.
After an exciting day of science yesterday, day 2 saw updates on strategies to rid cells of the harmful mutant huntingtin protein and exciting reports on current and planned clinical trials.