Premium
Necrosome‐positive granulovacuolar degeneration is associated with TDP‐43 pathological lesions in the hippocampus of ALS/FTLD cases
Author(s) -
Van Schoor E.,
Koper M. J.,
Ospitalieri S.,
Dedeene L.,
Tomé S. O.,
Vandenberghe R.,
Brenner D.,
Otto M.,
Weishaupt J.,
Ludolph A. C.,
Van Damme P.,
Van Den Bosch L.,
Thal D. R.
Publication year - 2021
Publication title -
neuropathology and applied neurobiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.538
H-Index - 95
eISSN - 1365-2990
pISSN - 0305-1846
DOI - 10.1111/nan.12668
Subject(s) - frontotemporal lobar degeneration , c9orf72 , amyotrophic lateral sclerosis , pathology , neurofibrillary tangle , hippocampus , frontotemporal dementia , hippocampal formation , spinal cord , biology , dementia , neuroscience , medicine , alzheimer's disease , disease , senile plaques
In ALS and FTLD‐TDP necrosome formation (= formation of a complex consisting of pRIPK1, pRIPK3 and pMLKL) is observed in granulovacuolar degeneration in neurons of the medial temporal lobe and correlates with TDP‐43 neuronal cytoplasmic inclusions. Motor neurons in the spinal cord and in the primary motor cortex do not show these granulovacuolar degeneration lesions. Accordingly, necrosome accumulation is one type of cell death pathology probably relevant in medial temporal lobe neurons of ALS and FTLD‐TDP cases but not in ALS‐related motor neuron death, for which another cell death mechanism may be responsible.