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
-
CADD Modeling of Multi-Target Drugs Against Alzheimer's Disease
Current Drug Targets The growth behavior of the model eukaryotic yeast Saccharomyces cerevisiae in microgravity
Current Biotechnology Nanotechnology for Alzheimer Disease
Current Alzheimer Research Melatonin Receptor Agonists: SAR and Applications to the Treatment of Sleep-Wake Disorders
Current Topics in Medicinal Chemistry Antioxidant Properties of Crocus Sativus L. and Its Constituents and Relevance to Neurodegenerative Diseases; Focus on Alzheimer’s and Parkinson’s Disease
Current Neuropharmacology Phosphoproteomics as a Promising Tool for Broadening the Analysis of Clinical Samples and for the Fight Against Cancer Disease
Current Pharmaceutical Analysis Functional Selectivity in Cannabinoid Signaling
Current Molecular Pharmacology Anti-Inflammatory Therapy for Alzheimer’s Disease from Epidemiological Fact to New Mechanisms of Action
Anti-Inflammatory & Anti-Allergy Agents in Medicinal Chemistry Sirtuin3 in Neurological Disorders
Current Drug Research Reviews Putative Molecular Interactions Involving Naturally Occurring Steroidal Alkaloids from Sarcococca hookeriana Against Acetyl- and Butyryl- Cholinesterase
Current Bioinformatics Docking-Related Survey on Natural-Product-Based New Monoamine Oxidase Inhibitors and Their Therapeutic Potential
Combinatorial Chemistry & High Throughput Screening Peptide Agonists and Antagonists with Potential Application in Neurological Disorders
Recent Patents on CNS Drug Discovery (Discontinued) Drusen in the Peripheral Retina of the Alzheimer’s Eye
Current Alzheimer Research GSK-3 Inhibitors: A New Class of Drugs for Alzheimer’s Disease Treatment
Current Drug Targets Immunotherapeutic Approaches in MS: Update on Pathophysiology and Emerging Agents or Strategies 2006
Endocrine, Metabolic & Immune Disorders - Drug Targets Therapeutic Approaches to Modulating Glutathione Levels as a Pharmacological Strategy in Alzheimer`s Disease
Current Alzheimer Research Cell Microencapsulation Implants into the Central Nervous System
Recent Patents on Nanomedicine Derivatives of 6-Nitrobenzimidazole Inhibit Fructose-Mediated Protein Glycation and Intracellular Reactive Oxygen Species Production
Medicinal Chemistry Therapeutic Strategies for Alzheimer's and Parkinson's Diseases by Means of Drug Delivery Systems
Current Medicinal Chemistry Mechanism of Action of Flavonoids as Anti-inflammatory Agents: A Review
Inflammation & Allergy - Drug Targets (Discontinued)