Neuroimmunology of Huntington’s Disease: Revisiting Evidence from Human Studies

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...

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Bibliographic Details
Published in:Mediators of inflammation 2016-01, Vol.2016 (2016), p.1-10
Main Authors: Teixeira, Antonio Lucio, Furr-Stimming, Erin, Ribeiro, Fabiola M., Rocha, Natália Pessoa
Format: Article
Language:English
Subjects:
Anti-Inflammatory Agents - therapeutic use
Brain
Central Nervous System - drug effects
Central Nervous System - immunology
Central Nervous System - metabolism
Central Nervous System - pathology
Cytokines
Cytokines - metabolism
Humans
Huntington Disease - drug therapy
Huntington Disease - immunology
Huntington Disease - metabolism
Huntington Disease - pathology
Huntington's chorea
Huntingtons disease
Inflammation
Inflammation - drug therapy
Inflammation - immunology
Inflammation - metabolism
Inflammation - pathology
Medical research
Medicine, Experimental
Nervous system
Neurodegeneration
Neuroimmunology
Neuropathology
Pathogenesis
Peripheral Nervous System - drug effects
Peripheral Nervous System - immunology
Peripheral Nervous System - metabolism
Peripheral Nervous System - pathology
Physiological aspects
Proteins
Review
Studies
Tumor necrosis factor-TNF
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Summary: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.
ISSN:0962-9351
1466-1861
DOI:10.1155/2016/8653132