title: Neuroimmunology of Huntington's Disease: Revisiting Evidence from Human Studies.
abstract: Huntington's disease (HD) is a neurodegenerative disorder characterized by selective loss of neurons in the striatum and cortex, which leads to progressive motor dysfunction, cognitive decline, and psychiatric disorders. Although the cause of HD is well described-HD is a genetic disorder caused by a trinucleotide (CAG) repeat expansion in the gene encoding for huntingtin (HTT) on chromosome 4p16.3-the ultimate cause of neuronal death is still uncertain. Apart from impairment in systems for handling abnormal proteins, other metabolic pathways and mechanisms might contribute to neurodegeneration and progression of HD. Among these, inflammation seems to play a role in HD pathogenesis. The current review summarizes the available evidence about immune and/or inflammatory changes in HD. HD is associated with increased inflammatory mediators in both the central nervous system and periphery. Accordingly, there have been some attempts to slow HD progression targeting the immune system.
authors: Neuropsychiatry Program, Department of Psychiatry and Behavioral Sciences, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX 77054, USA.
authors: Natalia P
authors: 0000-0003-2616-8082
authors: NP
authors: Rocha
authors: Department of Biochemistry and Immunology, ICB, Universidade Federal de Minas Gerais, 31270-901 Belo Horizonte, MG, Brazil.
authors: Fabiola M
authors: FM
authors: Ribeiro
authors: Department of Neurology, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX 77030, USA.
authors: Erin
authors: E
authors: Furr-Stimming
authors: Neuropsychiatry Program, Department of Psychiatry and Behavioral Sciences, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX 77054, USA.
authors: Antonio L
authors: 0000-0002-9621-5422
authors: AL
authors: Teixeira
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002490:Central Nervous System / Q000187:drug effects / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D016207:Cytokines / Q000378:metabolism; D006801:Humans; D006816:Huntington Disease / Q000188:drug therapy / Q000276:immunology* / Q000378:metabolism* / Q000473:pathology; D007249:Inflammation / Q000188:drug therapy / Q000276:immunology* / Q000378:metabolism* / Q000473:pathology; D017933:Peripheral Nervous System / Q000187:drug effects / Q000276:immunology / Q000378:metabolism / Q000473:pathology
chemical_list: D000893:Anti-Inflammatory Agents; D016207:Cytokines