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- W3211117521 abstract "TAR-DNA-binding protein of 43 kDa (TDP-43) has been found in an unstable aggregated form in patients suffering from the inherited form of amyotrophic lateral sclerosis (ALS) and frontal temporal lobar degeneration and has also been associated with Alzheimer's, Parkinson's, and Huntington's diseases. The interaction of a fraction of TDP-43 bound with fused in sarcoma/translocation in liposarcoma (FUS/TLS) is considerably enhanced by TDP-43 mutants. The abnormal stability of ALS-linked mutant TDP-43 and its increased binding to normal FUS/TLS implicates its pathogenic role in ALS. The C-terminal domain and N-terminal domain (NTD) are found to play a role in the pathological function of TDP-43. NTD also plays an important part in its biological functions. We have used the system biology approach to highlight the significance of TDP-43 in ALS and other neurodegenerative disorders. Expression, network, enrichment, and other analyses were done and the results of these in silico studies supported our experimentally conceived notion of importance and pathogenicity of TDP-43. The results show the ominous presence of TDP-43 in all tissues of the brain and its association with several neurological and muscular disorders." @default.
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- W3211117521 date "2022-01-01" @default.
- W3211117521 modified "2023-10-01" @default.
- W3211117521 title "A systems biology approach to understand the role of TDP-43 in amyotrophic lateral sclerosis" @default.
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- W3211117521 doi "https://doi.org/10.1016/b978-0-12-820066-7.00006-0" @default.
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