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We then genotyped the <jats:italic>TREM2<\/jats:italic> R47H gene variant and 3 <jats:italic>TLR4<\/jats:italic> single nucleotide polymorphisms in a large cohort of HD patients from the European Huntington's Disease Network REGISTRY.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We found an increase in the number of cells expressing TREM2 and TLR4 in postmortem brain samples from patients dying with HD. We also found that the <jats:italic>TREM2<\/jats:italic> R47H gene variant was associated with changes in cognitive decline in the large cohort of HD patients, whereas 2 of 3 <jats:italic>TLR4<\/jats:italic> single nucleotide polymorphisms assessed were associated with changes in motor progression in this same group.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These findings identify <jats:italic>TREM2<\/jats:italic> and <jats:italic>TLR4<\/jats:italic> as potential genetic modifiers for HD and suggest that inflammation influences disease progression in this condition. \u00a9 2019 International Parkinson and Movement Disorder Society<\/jats:p><\/jats:sec>","DOI":"10.1002\/mds.27911","type":"journal-article","created":{"date-parts":[[2019,11,14]],"date-time":"2019-11-14T03:55:24Z","timestamp":1573703724000},"page":"401-408","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Association Between Toll\u2010Like Receptor 4 (<i>TLR4<\/i>) and Triggering Receptor Expressed on Myeloid Cells 2 (<i>TREM2<\/i>) Genetic Variants and Clinical Progression of Huntington's Disease"],"prefix":"10.1002","volume":"35","author":[{"given":"Romina","family":"Vuono","sequence":"first","affiliation":[{"name":"John van Geest Centre for Brain Repair &amp; Department of Neurology, Department of Clinical Neurosciences University of Cambridge  Cambridge United Kingdom"},{"name":"Medway School of Pharmacy University of Kent at Medway  Kent United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonina","family":"Kouli","sequence":"additional","affiliation":[{"name":"John van Geest Centre for Brain Repair &amp; Department of Neurology, Department of Clinical Neurosciences University of Cambridge  Cambridge United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emilie M.","family":"Legault","sequence":"additional","affiliation":[{"name":"Faculty of Pharmacy University of Montreal  Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauriane","family":"Chagnon","sequence":"additional","affiliation":[{"name":"Faculty of Pharmacy University of Montreal  Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kieren S.","family":"Allinson","sequence":"additional","affiliation":[{"name":"Department of Pathology Cambridge University Hospitals NHS (National Health Service) Foundation Trust  Cambridge United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"La Spada","sequence":"additional","affiliation":[{"name":"Integrated Systems Engineering  Milano Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"REGISTRY Investigators of the European Huntington's Disease Network","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ida","family":"Biunno","sequence":"additional","affiliation":[{"name":"Institute for Genetic and Biomedical Research \u2010 CNR  Milano Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger A.","family":"Barker","sequence":"additional","affiliation":[{"name":"John van Geest Centre for Brain Repair &amp; Department of Neurology, Department of Clinical Neurosciences University of Cambridge  Cambridge United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janelle","family":"Drouin\u2010Ouellet","sequence":"additional","affiliation":[{"name":"Faculty of Pharmacy University of Montreal  Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,11,14]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/306234a0"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0893-387"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0893-393"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0893-398"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/2.10.1547"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.31.5.377"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00209410"},{"key":"e_1_2_8_9_1","first-page":"593","article-title":"Anticipation and instability of IT\u201015 (CAG)n repeats in parent\u2010offspring pairs with Huntington disease","volume":"57","author":"Ranen NG1","year":"1995","journal-title":"Am J Hum Genet"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.46.2.527"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.64.6.758"},{"key":"e_1_2_8_12_1","first-page":"1202","article-title":"The likelihood of being affected with Huntington disease by a particular age","volume":"60","author":"Brinkman RR","year":"1997","journal-title":"for a specific CAG size. 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