Title |
Proteostasis in striatal cells and selective neurodegeneration in Huntington’s disease
|
---|---|
Published in |
Frontiers in Cellular Neuroscience, August 2014
|
DOI | 10.3389/fncel.2014.00218 |
Pubmed ID | |
Authors |
Julia Margulis, Steven Finkbeiner |
Abstract |
Selective neuronal loss is a hallmark of neurodegenerative diseases, including Huntington's disease (HD). Although mutant huntingtin, the protein responsible for HD, is expressed ubiquitously, a subpopulation of neurons in the striatum is the first to succumb. In this review, we examine evidence that protein quality control pathways, including the ubiquitin proteasome system, autophagy, and chaperones, are significantly altered in striatal neurons. These alterations may increase the susceptibility of striatal neurons to mutant huntingtin-mediated toxicity. This novel view of HD pathogenesis has profound therapeutic implications: protein homeostasis pathways in the striatum may be valuable targets for treating HD and other misfolded protein disorders. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
United States | 1 | 50% |
Switzerland | 1 | 50% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 2 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Austria | 2 | 3% |
Portugal | 1 | 1% |
Germany | 1 | 1% |
United Kingdom | 1 | 1% |
Belgium | 1 | 1% |
United States | 1 | 1% |
Unknown | 63 | 90% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Ph. D. Student | 18 | 26% |
Researcher | 12 | 17% |
Student > Bachelor | 10 | 14% |
Student > Master | 9 | 13% |
Student > Doctoral Student | 3 | 4% |
Other | 8 | 11% |
Unknown | 10 | 14% |
Readers by discipline | Count | As % |
---|---|---|
Agricultural and Biological Sciences | 18 | 26% |
Neuroscience | 18 | 26% |
Biochemistry, Genetics and Molecular Biology | 9 | 13% |
Medicine and Dentistry | 9 | 13% |
Psychology | 2 | 3% |
Other | 3 | 4% |
Unknown | 11 | 16% |