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Lookup NU author(s): Emeritus Professor Robert Perry, Dr Evelyn Jaros, Professor David Burn, Professor Ian McKeith
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We report a new disease, dementia with neurofilament inclusions, characterized clinically by early-onset dementia with frontal lobe signs, focal atrophy of the frontal and temporal lobes, and microscopically by the presence in many brain regions of intraneuronal, cytoplasmic, neurofilament inclusions. The neuronal inclusions are immunoreactive to all three molecular weight neurofilament subunits: heavy (NF-H), light, and medium subunits, including the phosphorylated and non-phosphorylated forms of NF-H. Prion protein and beta-amyloid deposits were absent. The inclusions do not contain tau or alpha-synuclein protein aggregates known to characterize many neurodegenerative disorders. In addition to delineating a new disease entity, the identification of intraneuronal, cytoplasmic, neurofilament inclusions extends the molecular classification of neurodegenerative diseases and implicates new mechanisms of neurodegeneration in diseases affecting the human brain. (C) 2003 Elsevier Science Ireland Ltd. All rights reserved.
Author(s): Cairns NJ, Perry RH, Jaros E, Burn D, McKeith IG, Lowe JS, Holton J, Rossor MN, Skullerud K, Duyckaerts C, Cruz-Sanchez FF, Lantos PL
Publication type: Article
Publication status: Published
Journal: Neuroscience Letters
Year: 2003
Volume: 341
Issue: 3
Pages: 177-180
ISSN (print): 0304-3940
ISSN (electronic): 1872-7972
Publisher: Elsevier
URL: http://dx.doi.org/10.1016/S0304-3940(03)00100-9
DOI: 10.1016/S0304-3940(03)00100-9
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