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- W2800851630 abstract "Abstract The transactivation response DNA binding protein (TDP-43) is an RNA-binding protein involved in the processing of hundreds to thousands of RNA transcripts. This protein becomes mislocalized to the cytoplasm and forms characteristic inclusions found in most cases of amyotrophic lateral sclerosis (ALS) and many cases of frontotemporal dementia. Rare mutations in the gene encoding TDP-43 also cause ALS. Herein, we briefly review the genetics, pathology, and molecular and cellular basis of ALS, with a particular focus on TDP-43 and related TDP-43 proteinopathies. Dysfunctional TDP-43 can affect multiple cellular pathways, leading to cellular stress and neurodegeneration. Further elucidation of the normal and pathological role of TDP-43 may reveal strategies for the development of effective therapeutics." @default.
- W2800851630 created "2018-05-17" @default.
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- W2800851630 date "2018-01-01" @default.
- W2800851630 modified "2023-09-26" @default.
- W2800851630 title "Amyotrophic Lateral Sclerosis and Other TDP-43 Proteinopathies" @default.
- W2800851630 doi "https://doi.org/10.1016/b978-0-12-811304-2.00005-5" @default.
- W2800851630 hasPublicationYear "2018" @default.
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