The Role of the Immune System in Huntington’s Disease
Huntington’s disease (HD) is characterized by a progressive course of disease until death 15–20 years after the first symptoms occur and is caused by a mutation with expanded CAG repeats in the huntingtin (htt) protein. Mutant htt (mhtt) in the striatum is assumed to be the main reason for neurodege...
Saved in:
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2013-01-01
|
| Series: | Clinical and Developmental Immunology |
| Online Access: | http://dx.doi.org/10.1155/2013/541259 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832565128747286528 |
|---|---|
| author | Gisa Ellrichmann Christiane Reick Carsten Saft Ralf A. Linker |
| author_facet | Gisa Ellrichmann Christiane Reick Carsten Saft Ralf A. Linker |
| author_sort | Gisa Ellrichmann |
| collection | DOAJ |
| description | Huntington’s disease (HD) is characterized by a progressive course of disease until death 15–20 years after the first symptoms occur and is caused by a mutation with expanded CAG repeats in the huntingtin (htt) protein. Mutant htt (mhtt) in the striatum is assumed to be the main reason for neurodegeneration. Knowledge about pathophysiology has rapidly improved discussing influences of excitotoxicity, mitochondrial damage, free radicals, and inflammatory mechanisms. Both innate and adaptive immune systems may play an important role in HD. Activation of microglia with expression of proinflammatory cytokines, impaired migration of macrophages, and deposition of complement factors in the striatum indicate an activation of the innate immune system. As part of the adaptive immune system, dendritic cells (DCs) prime T-cell responses secreting inflammatory mediators. In HD, DCs may contain mhtt which brings the adaptive immune system into the focus of interest. These data underline an increasing interest in the peripheral immune system for pathomechanisms of HD. It is still unclear if neuroinflammation is a reactive process or if there is an active influence on disease progression. Further understanding the influence of inflammation in HD using mouse models may open various avenues for promising therapeutic approaches aiming at slowing disease progression or forestalling onset of disease. |
| format | Article |
| id | doaj-art-cb0238262c9a43a3932580e49f225ec3 |
| institution | Kabale University |
| issn | 1740-2522 1740-2530 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Clinical and Developmental Immunology |
| spelling | doaj-art-cb0238262c9a43a3932580e49f225ec32025-02-03T01:09:13ZengWileyClinical and Developmental Immunology1740-25221740-25302013-01-01201310.1155/2013/541259541259The Role of the Immune System in Huntington’s DiseaseGisa Ellrichmann0Christiane Reick1Carsten Saft2Ralf A. Linker3Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, 44791 Bochum, GermanyDepartment of Neurology, St. Josef-Hospital, Ruhr-University Bochum, 44791 Bochum, GermanyDepartment of Neurology, St. Josef-Hospital, Ruhr-University Bochum, 44791 Bochum, GermanyDepartment of Neurology, Friedrich-Alexander-University Erlangen, 91054 Erlangen, GermanyHuntington’s disease (HD) is characterized by a progressive course of disease until death 15–20 years after the first symptoms occur and is caused by a mutation with expanded CAG repeats in the huntingtin (htt) protein. Mutant htt (mhtt) in the striatum is assumed to be the main reason for neurodegeneration. Knowledge about pathophysiology has rapidly improved discussing influences of excitotoxicity, mitochondrial damage, free radicals, and inflammatory mechanisms. Both innate and adaptive immune systems may play an important role in HD. Activation of microglia with expression of proinflammatory cytokines, impaired migration of macrophages, and deposition of complement factors in the striatum indicate an activation of the innate immune system. As part of the adaptive immune system, dendritic cells (DCs) prime T-cell responses secreting inflammatory mediators. In HD, DCs may contain mhtt which brings the adaptive immune system into the focus of interest. These data underline an increasing interest in the peripheral immune system for pathomechanisms of HD. It is still unclear if neuroinflammation is a reactive process or if there is an active influence on disease progression. Further understanding the influence of inflammation in HD using mouse models may open various avenues for promising therapeutic approaches aiming at slowing disease progression or forestalling onset of disease.http://dx.doi.org/10.1155/2013/541259 |
| spellingShingle | Gisa Ellrichmann Christiane Reick Carsten Saft Ralf A. Linker The Role of the Immune System in Huntington’s Disease Clinical and Developmental Immunology |
| title | The Role of the Immune System in Huntington’s Disease |
| title_full | The Role of the Immune System in Huntington’s Disease |
| title_fullStr | The Role of the Immune System in Huntington’s Disease |
| title_full_unstemmed | The Role of the Immune System in Huntington’s Disease |
| title_short | The Role of the Immune System in Huntington’s Disease |
| title_sort | role of the immune system in huntington s disease |
| url | http://dx.doi.org/10.1155/2013/541259 |
| work_keys_str_mv | AT gisaellrichmann theroleoftheimmunesysteminhuntingtonsdisease AT christianereick theroleoftheimmunesysteminhuntingtonsdisease AT carstensaft theroleoftheimmunesysteminhuntingtonsdisease AT ralfalinker theroleoftheimmunesysteminhuntingtonsdisease AT gisaellrichmann roleoftheimmunesysteminhuntingtonsdisease AT christianereick roleoftheimmunesysteminhuntingtonsdisease AT carstensaft roleoftheimmunesysteminhuntingtonsdisease AT ralfalinker roleoftheimmunesysteminhuntingtonsdisease |