Journal
BIOCHEMICAL SOCIETY TRANSACTIONS
Volume 39, Issue -, Pages 954-959Publisher
PORTLAND PRESS LTD
DOI: 10.1042/BST0390954
Keywords
amyotrophic lateral sclerosis (ALS); animal model; frontotemporal lobar degeneration (FTLD); TAR DNA-binding protein 43 (TDP-43)
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Funding
- Belgian Federal Science Policy Office [P6/43]
- Medical Foundation Queen Elisabeth
- Foundation for Alzheimer Research (SAO/FRMA)
- Flemish Government
- Research Foundation Flanders
- Flemish Agency for Innovation by Science and Technology
- University of Antwerp
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TDP-43 (TAR DNA-binding protein 43) has been identified as a key protein of ubiquitinated inclusions in brains of patients with ALS (amyotrophic lateral sclerosis) or FTLD (frontotemporal lobar degeneration), defining a new pathological disease spectrum. Recently, coding mutations have been identified in the TDP-43 gene (TARDBP), which further confirmed the pathogenic nature of the protein. Today, several animal models have been generated to gain more insight into the disease-causing pathways of the FTLD/ALS spectrum. This mini-review summarizes the current status of TDP-43 models, with a focus on mutant TDP-43.
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