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TAR-DNA binding protein 43 in Pick disease.

AbstractPick disease (PiD) is a frontotemporal dementia characterized by frontal and temporal atrophy, neuronal loss, gliosis, ballooned neurons that are positive for alpha-B 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 immunohistochemical 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.
AuthorsStefanie H Freeman, Tara Spires-Jones, Bradley T Hyman, John H Growdon, Matthew P Frosch (Affiliation: Department of Pathology, MassGeneral Institute for Neurodegenerative Disease, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02129, USA. sfreeman1 at partners.org)
JournalJournal of neuropathology and experimental neurology (J Neuropathol Exp Neurol) Vol. 67 Issue 1 Pg. 62-7 (Jan 2008) ISSN: 0022-3069 United States
PMID18091558 (Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
Chemical References
  • DNA-Binding Proteins
  • Ubiquitin
  • protein TDP-43
  • tau Proteins
Topics
  • Aged
  • Aged, 80 and over
  • Brain (metabolism, pathology)
  • Case-Control Studies
  • DNA-Binding Proteins (metabolism)
  • Female
  • Humans
  • Intranuclear Inclusion Bodies (metabolism)
  • Male
  • Middle Aged
  • Neurons (metabolism, pathology)
  • Pick Disease of the Brain (metabolism, pathology)
  • Ubiquitin (metabolism)
  • tau Proteins (metabolism)