title: [A new dementia group caused by TDP-43 abnormality].
abstract: The TAR DNA-binding protein Mr 43 kDa (TDP-43) is a major component of the tau-negative and ubiquitin-positive inclusions that characterize amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration, which is now referred to as FTLD-TDP. Concurrent TDP-43 pathology has been reported in a variety of other neurodegenerative disorders such as Alzheimer's disease, forming a group of TDP-43 proteinopathies. Accumulated TDP-43 is characterized by phosphorylation and fragmentation. There is a close relationship between the pathological subtypes of FTLD-TDP and the immunoblot pattern of the C-terminal fragments of phosphorylated TDP-43. These results suggest that proteolytic processing of accumulated TDP-43 may play an important role in the pathological process. Understanding the mechanism of phosphorylation and truncation of TDP-43, and aggregate formation, may be crucial to clarifying the pathogenesis of TDP-43 proteinopathy and for developing useful therapies.
authors.affiliation: Department of Psychiatry, Graduate School of Comprehensive Human Sciences, University of Tsukuba.
authors.forename: Tetsuaki
authors.initials: T
authors.lastname: Arai
authors.forename: Masato
authors.initials: M
authors.lastname: Hosokawa
authors.forename: Masato
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Akiyama
authors.forename: Takashi
authors.initials: T
authors.lastname: Asada
mesh_terms: D004268:DNA-Binding Proteins / Q000378:metabolism; D006801:Humans; D057177:TDP-43 Proteinopathies / Q000235:genetics / Q000378:metabolism*
chemical_list: D004268:DNA-Binding Proteins