Title |
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice
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Published in |
Molecular Neurodegeneration, October 2011
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DOI | 10.1186/1750-1326-6-73 |
Pubmed ID | |
Authors |
Ya-Fei Xu, Yong-Jie Zhang, Wen-Lang Lin, Xiangkun Cao, Caroline Stetler, Dennis W Dickson, Jada Lewis, Leonard Petrucelli |
Abstract |
Abnormal distribution, modification and aggregation of transactivation response DNA-binding protein 43 (TDP-43) are the hallmarks of multiple neurodegenerative diseases, especially frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) and amyotrophic lateral sclerosis (ALS). Researchers have identified 44 mutations in the TARDBP gene that encode TDP-43 as causative for cases of sporadic and familial ALS http://www.molgen.ua.ac.be/FTDMutations/. Certain mutant forms of TDP-43, such as M337V, are associated with increased low molecular weight (LMW) fragments compared to wild-type (WT) TDP-43 and cause neuronal apoptosis and developmental delay in chick embryos. Such findings support a direct link between altered TDP-43 function and neurodegeneration. |
X Demographics
Geographical breakdown
Country | Count | As % |
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Unknown | 1 | 100% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 1 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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Germany | 1 | <1% |
United Kingdom | 1 | <1% |
Belgium | 1 | <1% |
Spain | 1 | <1% |
United States | 1 | <1% |
Unknown | 195 | 98% |
Demographic breakdown
Readers by professional status | Count | As % |
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Student > Ph. D. Student | 47 | 24% |
Student > Bachelor | 30 | 15% |
Researcher | 28 | 14% |
Student > Master | 24 | 12% |
Student > Doctoral Student | 9 | 5% |
Other | 23 | 12% |
Unknown | 39 | 20% |
Readers by discipline | Count | As % |
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Neuroscience | 39 | 20% |
Biochemistry, Genetics and Molecular Biology | 23 | 12% |
Medicine and Dentistry | 20 | 10% |
Nursing and Health Professions | 4 | 2% |
Other | 7 | 4% |
Unknown | 44 | 22% |