4.3 Article

TAR-DNA binding protein 43 in Pick disease

Journal

Publisher

LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/nen.0b013e3181609361

Keywords

frontotemporal dementia; immunohistochemistry; neurodegeneration; Pick bodies; Pick disease; TDP-43

Funding

  1. NIA NIH HHS [AG05134] Funding Source: Medline
  2. NATIONAL INSTITUTE ON AGING [P50AG005134] Funding Source: NIH RePORTER

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Pick disease (PiD) is a frontotemporal dementia characterized by frontal and temporal atrophy, neuronal loss, gliosis, ballooned neurons that are positive for alpha-13 crystallin and neurofilament, and the presence of tau- and ubiquitin-positive Pick bodies. TAR-DNA binding protein 43 (TDP-43) has been found to be a component of ubiquitinated inclusions in other neurodegenerative diseases, including frontotemporal lobar degeneration with ubiquitinated inclusions and amyotrophic lateral sclerosis. Fifteen cases of PiD were examined using imimmohistochemical methods, and 5 cases with both Pick bodies and smaller intracytoplasmic inclusions that showed staining for ubiquitin, tau, and TDP-43 were observed. The presence of TDP-43 inclusions in PiD suggests that TDP-43 accumulation may be an important component of many neurodegenerative diseases, and that its presence in only some cases of PiD may indicate different pathways of disease development.

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