【摘 要】
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TAR DNA-binding protein 43 (TDP-43) is an essential 414 amino acid protein that regulates multiple aspects of RNA biogenesis, processing, and transport. It localizes primarily in the nucleus, but abnormal translocation and accumulation in the cytosol occu
【机 构】
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Department of Neurology,McKnight Brain Institute,and Norman Fixel Institute for Neurological Disease
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TAR DNA-binding protein 43 (TDP-43) is an essential 414 amino acid protein that regulates multiple aspects of RNA biogenesis, processing, and transport. It localizes primarily in the nucleus, but abnormal translocation and accumulation in the cytosol occur under pathological conditions (Tziortzouda et al., 2021). TDP-43 abnormalities are typical pathological hallmarks of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. Mutations in the TDP-43-encoding gene TARDBP cause familial ALS, while wild-type TDP-43 is associated with almost all (～97％) of sporadic ALS cases and nearly half of frontotemporal lobar degeneration patients (～45％) (de Boer et al., 2020).
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