FUS pathology in basophilic inclusion body disease

@article{Munoz2009FUSPI,
  title={FUS pathology in basophilic inclusion body disease},
  author={David G Munoz and Manuela Neumann and Hirofumi Kusaka and Osamu Yokota and Kenji Ishihara and Seishi Terada and Shigetoshi Kuroda and Ian R A Mackenzie},
  journal={Acta Neuropathologica},
  year={2009},
  volume={118},
  pages={617-627}
}
Basophilic Inclusion Body Disease (BIBD) is a tau-negative form of frontotemporal lobar degeneration (FTLD), characterized by neuronal cytoplasmic inclusions (NCI) that are visible on hematoxylin and eosin stain (HE), contain RNA, and are inconsistently ubiquitin-immunoreactive (ir). The normal nuclear expression of TDP-43 is not altered. Here we investigate whether the distribution of the structurally and functionally related protein fused in sarcoma (FUS) is altered in BIBD. Mutations in the… 
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TLDR
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TLDR
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Chorea as a clinical feature of the basophilic inclusion body disease subtype of fused-in-sarcoma-associated frontotemporal lobar degeneration
TLDR
Three cases of the behavioral variant of frontotemporal dementia (bvFTD) that display chorea with fused in sarcoma (FUS)-positive inclusions (FTLD-FUS) and the basophilic inclusion body disease (BIBD) subtype are identified.
The spectrum and severity of FUS-immunoreactive inclusions in the frontal and temporal lobes of ten cases of neuronal intermediate filament inclusion disease
TLDR
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Novel Types of Frontotemporal Lobar Degeneration: Beyond Tau and TDP-43
TLDR
The discovery that the pathological changes in atypical FTLD with ubiquitinated inclusions, neuronal intermediate filament inclusion disease, and basophilic inclusion body disease are immunoreactive for the fused in sarcoma (FUS), resulting in the creation of a new molecular subgroup (FTLD-FUS).
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