title: Disruption of immune cell function by mutant huntingtin in Huntington's disease pathogenesis.
abstract: Innate immune dysfunction is increasingly recognised as a key characteristic of neurodegenerative disease. In the fatal inherited neurological disorder, Huntington's disease, altered innate immune cell function and increased inflammation are observed in the brain and the periphery of disease gene carriers many years before symptom onset, suggesting a potentially early and important role in disease pathogenesis. This is due, at least in part, to the intrinsic effects of the disease-causing protein, mutant huntingtin, expressed in innate immune cells themselves. Understanding whether such innate immune dysfunction in Huntington's disease can be targeted to slow the onset and/or the progression of the disease has significant therapeutic implications and is the subject of much current research.
authors.affiliation: UCL Institute of Neurology, Department of Neurodegenerative Disease, London, UK.
authors.forename: Ralph
authors.initials: R
authors.lastname: Andre
authors.affiliation: UCL Institute of Neurology, Department of Neurodegenerative Disease, London, UK.
authors.forename: Lucy
authors.initials: L
authors.lastname: Carty
authors.affiliation: UCL Institute of Neurology, Department of Neurodegenerative Disease, London, UK. Electronic address: s.tabrizi@ucl.ac.uk.
authors.forename: Sarah J
authors.initials: SJ
authors.lastname: Tabrizi
mesh_terms: D000818:Animals; D002490:Central Nervous System / Q000276:immunology; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000235:genetics* / Q000276:immunology*; D007113:Immunity, Innate; D007249:Inflammation / Q000276:immunology; D017628:Microglia / Q000276:immunology; D009154:Mutation; D022423:Myeloid Cells / Q000276:immunology; D009419:Nerve Tissue Proteins / Q000235:genetics* / Q000276:immunology*
chemical_list: C086055:HTT protein, human; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins