Huntington's disease: from molecular pathogenesis to clinical treatment
@article{Ross2011HuntingtonsDF, title={Huntington's disease: from molecular pathogenesis to clinical treatment}, author={Christopher A. Ross and Sarah J. Tabrizi}, journal={The Lancet Neurology}, year={2011}, volume={10}, pages={83-98} }
1,277 Citations
Huntington's disease: a clinical review
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Antisense oligonucleotide therapy is one such approach with clinical trials currently under way that may bring us one step closer to treating and potentially preventing this devastating condition.
Clinical Features of Huntington's Disease.
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The epidemiology, genetics and pathogenesis of HD as well as the clinical presentation and management of HD, which is currently focused on symptomatic treatment are discussed.
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Two articles in this issue of Brain highlight the Huntington's disease research community's multi-faceted approach to developing new treatments for Huntington’s disease.
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It is suggested that HDL2 is a complex disease in which transcripts and proteins expressed bidirectionally from the junctophilin-3 locus contribute to pathogenesis through both gain-and loss-of-function mechanisms.
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- 2014
The preclinical and clinical development molecular therapies targeting HTT expression and the modulation of biological mechanisms thought to contribute to disease pathogenesis via novel therapeutic agents are reviewed.
Neuroimmunology of Huntington's Disease: Revisiting Evidence from Human Studies
- Biology, MedicineMediators of inflammation
- 2016
The current review summarizes the available evidence about immune and/or inflammatory changes in HD, which is associated with increased inflammatory mediators in both the central nervous system and periphery.
Molecular Mechanisms Underlying Neuropathology in Mouse Models of Huntington’s Disease
- Biology
- 2015
This dissertation will detail work performed in characterizing the cellular and molecular determinants of HD pathogenesis in novel mouse models developed in the Yang lab, using conditional genetics to define cells in the cortex and striatum as essential to developing HD phenotypes, and assessed molecular signatures in these cells that are dependent on the mutant huntingtin protein.
Huntington’s Disease: The Past, Present, and Future Search for Disease Modifiers
- BiologyThe Yale journal of biology and medicine
- 2013
Although silencing Htt expression appears promising as a disease modifying treatment, it should be attempted with caution in light of Htt’s essential roles in neural maintenance and development.
A Clinical Review on Huntington Disease
- Biology, MedicineGlobal Journal of Pharmacy & Pharmaceutical Sciences
- 2018
The mechanism by which neuronal degeneration and cell death are being generated in HD may include excitotoxicity, energy deficit, oxidative stress, inflammatory process and protein aggregation.
Preclinical and Clinical Investigations of Mood Stabilizers for Huntington's Disease: What Have We Learned?
- Biology, PsychologyInternational journal of biological sciences
- 2014
Preclinical and clinical investigations of the beneficial effects of lithium and VPA on HD pathophysiology are discussed, including behavioral and motor improvement, enhanced neuroprotection, and lifespan extension.
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