Publications

Authors:

Cagnin, Annachiara; Mariotto, Sara; Fiorini, Michele; Gaule, Marina; Bonetto, Nicola; Tagliapietra, Matteo; Buratti, Emanuele; Zanusso, Gianluigi; Ferrari, Sergio; Monaco, Salvatore

Title:

Microglial and Neuronal TDP-43 Pathology in Anti-IgLON5-Related Tauopathy

Year:

2017

Type of item:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Language:

Inglese

Format:

A Stampa

Referee:

Sì

Name of journal:

JOURNAL OF ALZHEIMER'S DISEASE

ISSN of journal:

1387-2877

N° Volume:

59

Number or Folder:

1

Page numbers:

13-20

Keyword:

IgLON5; TDP-43 pathology; microglia; non-cell autonomous neurodegeneration; tauopathy

Short description of contents:

A novel neuronal tauopathy, mainly confined to hypothalamus and brainstem tegmentum, has recently been reported in patients with autoantibodies to the neuronal cell-adhesion molecule IgLON5. We describe a patient with anti-IgLON5 syndrome, who presented with dysautonomia and sleep disorder, followed by subacute dementia. Postmortem brain examination disclosed neuronal tau pathology prevailing in the hippocampus, amygdala, and locus coeruleus, in addition to microglial/neuronal TDP-43 pathology, with overexpression of aberrantly phosphorylated forms and neurotoxic truncated fragments, in basal ganglia, nucleus basalis, thalamus, and midbrain. These findings suggest that neurodegeneration in anti-IgLON5 syndrome might also occur via a microglia-triggered non-cell autonomous pathway.

Web page:

https://doi.org/10.3233/JAD-170189

Product ID:

98524

Handle IRIS:

11562/966905

Last Modified:

November 26, 2022

Bibliographic citation:

Cagnin, Annachiara; Mariotto, Sara; Fiorini, Michele; Gaule, Marina; Bonetto, Nicola; Tagliapietra, Matteo; Buratti, Emanuele; Zanusso, Gianluigi; Ferrari, Sergio; Monaco, Salvatore, Microglial and Neuronal TDP-43 Pathology in Anti-IgLON5-Related Tauopathy «JOURNAL OF ALZHEIMER'S DISEASE» , vol. 59 , n. 1 , 2017 , pp. 13-20

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