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The role of immunity in Huntington's disease
1Axe Neurosciences, Centre de Recherche du CHUL (CHUQ), Boulevard Laurier, Québec, QC, Canada.
Insights
Huntington's disease (HD) involves more than the brain, with mutant huntingtin affecting the immune system. This review explores immunity's role in HD pathology and potential therapeutic strategies.
Area of Science:
- Neuroscience
- Immunology
- Genetics
Background:
- Huntington's disease (HD) is a neurodegenerative disorder with cognitive, psychiatric, and motor impairments.
- Emerging evidence suggests systemic abnormalities outside the central nervous system in HD patients.
- Mutant huntingtin (mHtt) is present in various cell types, interacting with the immune system.
Purpose of the Study:
- To provide a novel understanding of immune system interactions in HD pathology.
- To elucidate the potential pathogenic role of the immune system in Huntington's disease.
- To explore the beneficial or detrimental influence of immune system changes in HD.
Main Methods:
- Review of clinical data from anti-inflammatory drug trials in HD.
- Analysis of emerging evidence on mHtt interactions with the immune system.
- Proposal of new experimental approaches to investigate immunity in HD.
Main Results:
- Mutant huntingtin (mHtt) is found in multiple cell types, indicating systemic effects.
- The immune system's contribution to HD pathology is significant but not fully understood.
- Clinical trials of anti-inflammatory drugs in HD have yielded varied results.
Conclusions:
- The immune system plays a critical, yet unclear, role in Huntington's disease pathogenesis.
- Further research is needed to determine if immune modulation is beneficial or detrimental in HD.
- Novel experimental strategies are proposed to clarify immunity's role in this incurable disorder.
Abstract:
Huntington's disease (HD) is a devastating and incurable neurodegenerative disorder characterized by progressive cognitive, psychiatric and motor impairments. Although the disease has been seen as a disorder purely of the brain, there is now emerging evidence that abnormalities outside the central nervous system are commonly seen in HD. Indeed, the mutant huntingtin (mHtt) coded for by the abnormal gene in HD is found in every cell type where its presence has been sought. In particular, there are a number of recent observations in HD patients that mHtt interacts with the immune system with accumulating evidence that changes in the immune system may critically contribute to the pathology of HD. However, the nature of this contribution remains unclear, to the extent that it is not even known whether the immune system has a beneficial or detrimental role in HD patients. In this review, we attempt to bring a novel understanding to the interaction of the immune system to HD pathology, thereby shedding light on its potential pathogenic role. As part of this discussion, we revisit the clinical data on the anti-inflammatory drug trials in HD and propose new experimental approaches to interrogate the role of immunity in this currently incurable disorder.
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