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Title
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The role of mutant TAR DNA-binding protein 43 in amyotrophic lateral sclerosis and frontotemporal lobar degeneration
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Author
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Abstract
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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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Language
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English
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Source (journal)
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Biochemical Society transactions. - London, 1973, currens
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Publication
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London
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Biochemical Society
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2011
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ISSN
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0300-5127
[print]
1470-8752
[online]
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DOI
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10.1042/BST0390954
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Volume/pages
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39
:4
(2011)
, p. 954-959
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ISI
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000293814800019
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Full text (Publisher's DOI)
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