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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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    Title
    • HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation
    Author
    • Träger, Ulrike. 1 UCL Institute of Neurology, Department of Neurodegenerative Disease, London, UK
    • Andre, Ralph. 1 UCL Institute of Neurology, Department of Neurodegenerative Disease, London, UK
    • Lahiri, Nayana. 1 UCL Institute of Neurology, Department of Neurodegenerative Disease, London, UK
    • Magnusson-Lind, Anna. 1 UCL Institute of Neurology, Department of Neurodegenerative Disease, London, UK
    • Weiss, Andreas. 3 Novartis Institutes for BioMedical Research, Novartis Campus, Basel, Switzerland
    • Grueninger, Stephan. 3 Novartis Institutes for BioMedical Research, Novartis Campus, Basel, Switzerland
    • McKinnon, Chris. 1 UCL Institute of Neurology, Department of Neurodegenerative Disease, London, UK
    • Sirinathsinghji, Eva. 4 King's College London, Department of Medical and Molecular Genetics, Guy's Hospital, London, UK
    • Kahlon, Shira. 5 University of Massachusetts Medical School, Program in Molecular Medicine, Worcester, MA, USA
    • Pfister, Edith L.. 6 University of Massachusetts Medical School, Department of Medicine, Division Endocrinology and Metabolism, Worcester, MA, USA
    • Moser, Roger. 7 École Polytechnique Fédérale de Lausanne, Brain Mind Institute, Lausanne, Switzerland
    • Hummerich, Holger. 1 UCL Institute of Neurology, Department of Neurodegenerative Disease, London, UK
    • Antoniou, Michael. 4 King's College London, Department of Medical and Molecular Genetics, Guy's Hospital, London, UK
    • Bates, Gillian P.. 4 King's College London, Department of Medical and Molecular Genetics, Guy's Hospital, London, UK
    • Luthi-Carter, Ruth. 7 École Polytechnique Fédérale de Lausanne, Brain Mind Institute, Lausanne, Switzerland
    • Lowdell, Mark W.. 9 UCL, Department of Haematology, Royal Free Campus, London, UK
    • Björkqvist, Maria. 2 Wallenberg Neuroscience Centre, Department of Experimental Medical Science, Brain Disease Biomarker Unit, Lund University, Lund, Sweden
    • Ostroff, Gary R.. 5 University of Massachusetts Medical School, Program in Molecular Medicine, Worcester, MA, USA
    • Aronin, Neil. 6 University of Massachusetts Medical School, Department of Medicine, Division Endocrinology and Metabolism, Worcester, MA, USA
    • Tabrizi, Sarah J.. 1 UCL Institute of Neurology, Department of Neurodegenerative Disease, London, UK
    Document Type
    • Postprint
    OAI-PMH Identifier
    • oai:doc.rero.ch:299589
    Summary
    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