Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease

@article{Neumann2009AbundantFP,
  title={Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease},
  author={Manuela Neumann and Sigrun Roeber and Hans A. Kretzschmar and Rosa Rademakers and Matt C. Baker and Ian R A Mackenzie},
  journal={Acta Neuropathologica},
  year={2009},
  volume={118},
  pages={605-616}
}
Neuronal intermediate filament inclusion disease (NIFID) is an uncommon neurodegenerative condition that typically presents as early-onset, sporadic frontotemporal dementia (FTD), associated with a pyramidal and/or extrapyramidal movement disorder. The neuropathology is characterized by frontotemporal lobar degeneration with neuronal inclusions that are immunoreactive for all class IV intermediate filaments (IF), light, medium and heavy neurofilament subunits and α-internexin. However, not all… 

The spectrum and severity of FUS-immunoreactive inclusions in the frontal and temporal lobes of ten cases of neuronal intermediate filament inclusion disease

TLDR
A quantitative assessment of ten clinically and neuropathologically well-characterized cases using FUS IHC revealed significantly greater numbers of NCI in all brain regions especially the DG, and significantly more NCI revealed by FUS than α-internexin IHC.

Neuronal intermediate filament inclusion disease may be incorrectly classified as a subtype of FTLD-FUS.

TLDR
While FUS pathology remains a prominent feature of aFTLD-U, there is pathologic heterogeneity, including rare cases of NIFID with TDP-43- rather than FUS-positive inclusions.

Spatial patterns of FUS-immunoreactive neuronal cytoplasmic inclusions (NCI) in neuronal intermediate filament inclusion disease (NIFID)

TLDR
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TLDR
The above findings suggest that cytoplasmic mislocalization and aggregation of FUS appear at the early stage of the disease, and the FUS aggregate process may not be a direct precedent structure of HC.

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TLDR
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FUS‐Immunoreactive Intranuclear Inclusions in Neurodegenerative Disease

TLDR
In a cell culture model of Huntington's disease, NIIs were intensely FUS‐positive and NII‐bearing cells displayed loss of the normal diffuse nuclear pattern of FUS staining, suggesting that sequestration of nuclear FUS by NIIs may interfere with its normal nuclear localization.

Distinct pathological subtypes of FTLD-FUS

TLDR
Findings support aFTLD-U, NIFID and BIBD as representing closely related, but distinct entities that share a common molecular pathogenesis.

FUS Immunogold Labeling TEM Analysis of the Neuronal Cytoplasmic Inclusions of Neuronal Intermediate Filament Inclusion Disease: A Frontotemporal Lobar Degeneration with FUS Proteinopathy

TLDR
It is shown that FUS becomes relatively insoluble in NIFID and there is no apparent posttranslational modifications, there are no pathogenic abnormalities in the FUS gene in NifID, and immunoelectron microscopy demonstrates the fine structural localization of FUS in N IFID which has not previously been described.

Characteristic Features of FUS Inclusions in Spinal Motor Neurons of Sporadic Amyotrophic Lateral Sclerosis.

TLDR
A novel detection method using a conventional antigen retrieval technique with Sudan Black B treatment to identify FUS-positive inclusions in sALS patients and classified pathological motor neurons into 5 different categories according to the different aggregation characteristics of FUS and TDP-43.

Neuronal intermediate filament inclusion disease.

  • N. Cairns
  • Medicine, Biology
    Handbook of clinical neurology
  • 2008
...

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