HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation
Träger, Ulrike ; Andre, Ralph ; Lahiri, Nayana ; Magnusson-Lind, Anna ; Weiss, Andreas ; Grueninger, Stephan ; McKinnon, Chris ; Sirinathsinghji, Eva ; Kahlon, Shira ; Pfister, Edith L. ; Moser, Roger ; Hummerich, Holger ; Antoniou, Michael ; Bates, Gillian P. ; Luthi-Carter, Ruth ; Lowdell, Mark W. ; Björkqvist, Maria ; Ostroff, Gary R. ; Aronin, Neil ; Tabrizi, Sarah J.
In: Brain, 2014, vol. 137, no. 3, p. 819-833
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- The peripheral immune response is altered in Huntington's disease, but the underlying mechanisms are unclear. Using RNA interference to lower huntingtin levels in leucocytes from patients, Träger et al. reverse disease-associated phenotypes including cytokine elevation and transcriptional dysregulation, and argue for a direct effect of mutant huntingtin on NFκΒ signalling