Abstract
Huntingtons disease (HD) is a dominantly inherited neurodegenerative disorder caused by expansion of a polyglutamine (polyQ) tract in the huntingtin protein, resulting in intracellular aggregate formation and neurodegeneration. Biochemical pathways leading from polyQ expansion to disease pathogenesis are largely unknown. Recent approaches using genetic models systems have begun to uncover nuclear and cytoplasmic pathologies that represent potential targets for therapeutic intervention.
Keywords: Axonal transport, neurodegeneration, vesicle trafficking, protein aggregation, polyQ, huntington's disease
Mini-Reviews in Medicinal Chemistry
Title: Therapeutic Prospects for the Prevention of Neurodegeneration in Huntingtons Disease and the Polyglutamine Repeat Disorders
Volume: 7 Issue: 1
Author(s): Yoko Kimura, Wyan-Ching Mimi Lee and J. Troy Littleton
Affiliation:
Keywords: Axonal transport, neurodegeneration, vesicle trafficking, protein aggregation, polyQ, huntington's disease
Abstract: Huntingtons disease (HD) is a dominantly inherited neurodegenerative disorder caused by expansion of a polyglutamine (polyQ) tract in the huntingtin protein, resulting in intracellular aggregate formation and neurodegeneration. Biochemical pathways leading from polyQ expansion to disease pathogenesis are largely unknown. Recent approaches using genetic models systems have begun to uncover nuclear and cytoplasmic pathologies that represent potential targets for therapeutic intervention.
Export Options
About this article
Cite this article as:
Kimura Yoko, Mimi Lee Wyan-Ching and Troy Littleton J., Therapeutic Prospects for the Prevention of Neurodegeneration in Huntingtons Disease and the Polyglutamine Repeat Disorders, Mini-Reviews in Medicinal Chemistry 2007; 7 (1) . https://dx.doi.org/10.2174/138955707779317867
DOI https://dx.doi.org/10.2174/138955707779317867 |
Print ISSN 1389-5575 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5607 |
Call for Papers in Thematic Issues
Bioprospecting of Natural Products as Sources of New Multitarget Therapies
According to the Convention on Biological Diversity, bioprospecting is the exploration of biodiversity and indigenous knowledge to develop commercially valuable products for pharmaceutical and other applications. Bioprospecting involves searching for useful organic compounds in plants, fungi, marine organisms, and microorganisms. Natural products traditionally constituted the primary source of more than ...read more
Computational Frontiers in Medicinal Chemistry
The thematic issue "Computational Frontiers in Medicinal Chemistry" provides a robust platform for delving into state-of-the-art computational methodologies and technologies that significantly propel advancements in medicinal chemistry. This edition seeks to amalgamate top-tier reviews spotlighting the latest trends and breakthroughs in the fusion of computational approaches, including artificial intelligence (AI) ...read more
Drugs and mitochondria
Mitochondria play a central role in the life and death of cells. They are not merely the center for energy metabolism but are also the headquarters for different catabolic and anabolic processes, calcium fluxes, and various signaling pathways. Mitochondria maintain homeostasis in the cell by interacting with reactive oxygen-nitrogen species ...read more
Mitochondria as a Therapeutic Target in Metabolic Disorders
Mitochondria are the primary site of adenosine triphosphate (ATP) production in mammalian cells. Moreover, these organelles are an important source of reactive oxygen and nitrogen species in virtually any nucleated cell type. The modulation of a myriad of cellular signaling pathways depends on the mitochondrial physiology. Mitochondrial dysfunction is observed ...read more
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
Related Articles
-
H2O2 Signalling Pathway: A Possible Bridge between Insulin Receptor and Mitochondria
Current Neuropharmacology Pegylated Nanoparticles for Brain Targeting- Opportunities and Challenges
Current Nanomedicine Growth and Trophic Factors, pH and the Na+/H+ Exchanger in Alzheimers Disease, Other Neurodegenerative Diseases and Cancer: New Therapeutic Possibilities and Potential Dangers
Current Alzheimer Research Metabolic Syndrome and Cognitive Decline
Current Alzheimer Research Iron Chelating Strategies in Systemic Metal Overload, Neurodegeneration and Cancer
Current Medicinal Chemistry A Review of Computational Identification of Protein Post-Translational Modifications
Mini-Reviews in Organic Chemistry Is there a Rational Approach for Increasing Drug Specificity? Considerations on CNS Target Choice and Validation
Recent Patents on CNS Drug Discovery (Discontinued) LRRK2 and Parkinson's Disease: From Lack of Structure to Gain of Function
Current Protein & Peptide Science Toll-like Receptors as a Novel Therapeutic Target for Natural Products Against Chronic Diseases
Current Drug Targets Vascular Oxidative Stress and Mitochondrial Failure in the Pathobiology of Alzheimer’s Disease: A New Approach to Therapy
CNS & Neurological Disorders - Drug Targets NMDA Receptor Antagonists as Antidepressant and Antidementia Drugs: Recent Developments and Future Prospects
Current Medicinal Chemistry - Central Nervous System Agents Hunting for Peptide Substrates of Prolyl Oligopeptidase: Classical Versus Non-Classical Bioactive Peptides
CNS & Neurological Disorders - Drug Targets NMNAT Expression and its Relation to NAD Metabolism
Current Medicinal Chemistry Neuroprotective Methodologies of Co-Enzyme Q10 Mediated Brain Hemorrhagic Treatment: Clinical and Pre-Clinical Findings
CNS & Neurological Disorders - Drug Targets A Step Further Towards Multitarget Drugs for Alzheimer and Neuronal Vascular Diseases: Targeting the Cholinergic System, Amyloid-β Aggregation and Ca2++ Dyshomeostasis
Current Medicinal Chemistry Influence of Lithium Treatment on GDNF Serum and CSF Concentrations in Patients with Early Alzheimers Disease
Current Alzheimer Research Editorial [Hot Topic :Current Advances In Therapeutic Applications of Nuclear Receptors (Guest Editor: Stefano Fiorucci)]
Current Topics in Medicinal Chemistry Multiple Sclerosis – Established and Novel Therapeutic Approaches
Central Nervous System Agents in Medicinal Chemistry General Theory for Multiple Input-Output Perturbations in Complex Molecular Systems. 1. Linear QSPR Electronegativity Models in Physical, Organic, and Medicinal Chemistry
Current Topics in Medicinal Chemistry Functional Role of Lipoprotein Receptors in Alzheimers Disease
Current Alzheimer Research