TY - JOUR
T1 - Neuroimmunology of Huntington's Disease
T2 - Revisiting Evidence from Human Studies
AU - Rocha, Natalia P.
AU - Ribeiro, Fabiola M.
AU - Furr-Stimming, Erin
AU - Teixeira, Antonio L.
N1 - Publisher Copyright:
© 2016 Natalia P. Rocha et al.
PY - 2016
Y1 - 2016
N2 - 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.
AB - 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.
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U2 - 10.1155/2016/8653132
DO - 10.1155/2016/8653132
M3 - Review article
C2 - 27578922
AN - SCOPUS:84983762705
SN - 0962-9351
VL - 2016
JO - Mediators of Inflammation
JF - Mediators of Inflammation
M1 - 8653132
ER -