Abstract
Polyglutamine (polyQ) expansion causes nine inherited neurodegenerative disorders, including Huntingtons Disease, Spinobulbar Muscular Atrophy, Dentatorubral-Pallidoluysian Atrophy, and Spinocerebellar Ataxias 1, 2, 3, 6, 7, and 17. The common pathological feature of these diseases is the formation of intracellular polyglutamine inclusions or aggregates. Previous studies have focused on the intranuclear inclusions and found that polyQ proteins can affect gene transcription. Recent studies have revealed that polyQ proteins and their inclusions in neuronal processes can impair intracellular transport. Impaired intracellular trafficking, particularly in axons, may lead to neuronal dysfunction and early neuropathology. In this review, we will discuss the ways that polyQ proteins affect intracellular trafficking with an emphasis on the events that lead to neurodegeneration in Huntingtons disease.
Current Genomics
Title: Polyglutamine Protein Trafficking and Neurodegeneration
Volume: 6 Issue: 3
Author(s): John R. McGuire, Shi-Hua Li and Xiao-Jiang Li
Affiliation:
Abstract: Polyglutamine (polyQ) expansion causes nine inherited neurodegenerative disorders, including Huntingtons Disease, Spinobulbar Muscular Atrophy, Dentatorubral-Pallidoluysian Atrophy, and Spinocerebellar Ataxias 1, 2, 3, 6, 7, and 17. The common pathological feature of these diseases is the formation of intracellular polyglutamine inclusions or aggregates. Previous studies have focused on the intranuclear inclusions and found that polyQ proteins can affect gene transcription. Recent studies have revealed that polyQ proteins and their inclusions in neuronal processes can impair intracellular transport. Impaired intracellular trafficking, particularly in axons, may lead to neuronal dysfunction and early neuropathology. In this review, we will discuss the ways that polyQ proteins affect intracellular trafficking with an emphasis on the events that lead to neurodegeneration in Huntingtons disease.
Export Options
About this article
Cite this article as:
McGuire R. John, Li Shi-Hua and Li Xiao-Jiang, Polyglutamine Protein Trafficking and Neurodegeneration, Current Genomics 2005; 6 (3) . https://dx.doi.org/10.2174/1389202053971938
DOI https://dx.doi.org/10.2174/1389202053971938 |
Print ISSN 1389-2029 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5488 |
Call for Papers in Thematic Issues
Advanced Computational Algorithms and Artificial Intelligence in Clinical Pharmacogenomics
In the era of personalized medicine, understanding the relationship between genetics and drug response is crucial. This issue delves into innovative methodologies, leveraging deep computational analysis and artificial intelligence, to enhance the field of Clinical Pharmacogenomics. The interdisciplinary approach harnesses the power of advanced high-throughput genotyping technologies, sophisticated computational analysis, ...read more
Applications of Single-cell Sequencing Technology in Reproductive Medicine
Single cell sequencing (SCS) technology utilizes individual cells' genetic material to sequence their genome, transcriptome, and epigenetics at the molecular level. It offers insights into cell heterogeneity and enables the study of limited biological materials. Since its recognition as a valuable technique in 2011, single cell sequencing has yielded numerous ...read more
Big Data in Cancer Research
Cancer is a significant threat to human life and health, remaining a highly aggressive killer. It is a leading cause of death worldwide and represents a crucial medical issue for humanity. However, in the past decade, the effectiveness of new synthetic anticancer agents has not matched the current clinical speculation. ...read more
Current Genomics in Cardiovascular Research
Cardiovascular diseases are the main cause of death in the world, in recent years we have had important advances in the interaction between cardiovascular disease and genomics. In this Research Topic, we intend for researchers to present their results with a focus on basic, translational and clinical investigations associated with ...read more
Related Journals
- 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
- Announcements
Related Articles
-
Recent Approaches for Studying the Role of Glia
CNS & Neurological Disorders - Drug Targets Cancer Nanotechnology: Emerging Role of Gold Nanoconjugates
Anti-Cancer Agents in Medicinal Chemistry Pyroglutamate-Modified Amyloid Beta Peptides: Emerging Targets for Alzheimer´s Disease Immunotherapy
Current Neuropharmacology A MicroRNA-BDNF Negative Feedback Signaling Loop in Brain: Implications for Alzheimer’s Disease
MicroRNA Changing Paradigm from one Target one Ligand Towards Multi-target Directed Ligand Design for Key Drug Targets of Alzheimer Disease: An Important Role of In Silico Methods in Multi-target Directed Ligands Design
Current Neuropharmacology Silencing the Brain May be Better than Stimulating it. The GABA Effect
Current Pharmaceutical Design Advances in Studies on Neurodegenerative Diseases and their Treatments
Current Topics in Medicinal Chemistry The Role of Autophagy: What can be Learned from the Genetic Forms of Amyotrophic Lateral Sclerosis
CNS & Neurological Disorders - Drug Targets Proteasome Inhibitors Therapeutic Strategies for Cancer
Recent Patents on Anti-Cancer Drug Discovery Editorial (Thematic Issue: Multi-Target Drug Discovery in Medicinal Chemistry: Current Status and Future Perspectives)
Mini-Reviews in Medicinal Chemistry Patent Annotations
Recent Patents on CNS Drug Discovery (Discontinued) Drug Delivery Systems for Imaging and Therapy of Parkinson's Disease
Current Neuropharmacology Polyphenols and Aging
Current Aging Science In Silico Studies of (2E,5E)-2,5-bis(3-methoxy-4-hydroxy-benzylidene) Cyclopentanone on Proteins AChE and BChE Involved in Alzheimers disease and Ameliorative Effects on Paraquat Induced Oxidative Stress Markers in Drosophila melanogaster
Letters in Drug Design & Discovery Palmitoylethanolamide Regulates Production of Pro-Angiogenic Mediators in a Model of β Amyloid-Induced Astrogliosis <i>In Vitro</i>
CNS & Neurological Disorders - Drug Targets Targeting the Nogo-A Signalling Pathway to Promote Recovery Following Acute CNS Injury
Current Pharmaceutical Design Curcumin Activates the Nrf2 Pathway and Induces Cellular Protection Against Oxidative Injury
Current Molecular Medicine Inhibition of Human Serine Racemase, an Emerging Target for Medicinal Chemistry
Current Drug Targets Nanoparticle Enabled Drug Delivery Across the Blood Brain Barrier: in vivo and in vitro Models, Opportunities and Challenges
Current Pharmaceutical Biotechnology G-Protein Coupled Receptors Involved in the Resolution of Inflammation: Ligands and Therapeutic Perspectives
Mini-Reviews in Medicinal Chemistry