Publication

Subcellular clearance and accumulation of huntington disease protein: A mini-review

Downloadable Content

Persistent URL
Last modified
  • 02/20/2025
Type of Material
Authors
    Ting Zhao, Emory UniversityYan Hong, Emory UniversityXiao-Jiang Li, Emory UniversityShihua Li, Emory University
Language
  • English
Date
  • 2016-04-21
Publisher
  • Frontiers Media
Publication Version
Copyright Statement
  • © 2016 Zhao, Hong, Li and Li.
License
Final Published Version (URL)
Title of Journal or Parent Work
ISSN
  • 1662-5099
Volume
  • 9
Issue
  • APR
Grant/Funding Information
  • This work was supported by NIH Grants (AG19206 and NS041449 to X-JL, NS095279 and NS095181 to S-HL).
Abstract
  • Huntington’s disease (HD) is an autosomal dominant, progressive neurodegenerative disease caused by an expanded polyglutamine (polyQ) tract in the N-terminal region of mutant huntingtin (mHtt). As a result, mHtt forms aggregates that are abundant in the nuclei and processes of neuronal cells. Although the roles of mHtt aggregates are still debated, the formation of aggregates points to deficient clearance of mHtt in brain cells. Since the accumulation of mHtt is a prerequisite for its neurotoxicity, exploring the mechanisms for mHtt accumulation and clearance would advance our understanding of HD pathogenesis and help us develop treatments for HD. We know that the ubiquitinproteasome system (UPS) and autophagy play important roles in clearing mHtt; however, how mHtt preferentially accumulates in neuronal nuclei and processes remains unclear. Studying the clearance of mHtt in neuronal cells is a challenge because neurons are morphologically and functionally polarized, which means the turnover of mHtt may be distinct in different cellular compartments. In this review, we discuss our current knowledge about the clearance and accumulation of mHtt and strategies examining mHtt clearance and accumulation in different subcellular regions.
Author Notes
Keywords
Research Categories
  • Biology, Genetics
  • Biology, Neuroscience

Tools

Relations

In Collection:

Items

Thumbnail Title File Description Date Uploaded Visibility Actions
file details Publication File - rps7g.pdf Primary Content 2025-02-13 Public Download