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Introduction TDP-43 is a highly conserved and ubiquitously expressed 43 kDa protein that was first recognized as a TAR DNA binding protein of the LTR region of HIV-1, involved in the transcriptional repression of HIV-1 expression (Ou et al., 1995; Wang et al., 2004).
Introduction A broad spectrum of neurodegenerative conditions belongs to the class of TDP-43 proteinopathies. In all these conditions, TDP-43 is the main protein found in inclusions in the brain therefore linking all these diseases to this common pathological category. TDP-43 proteinopathy is found in ALS, FTD, Alzheimer’s disease (AD), and limbic-predominant age-related TDP-43 encephalopathy (LATE) (Nelson et al., 2019; Prasad et al., 2019).
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