Publication: A morphometric study of the spatial
patterns of TDP-43 immunoreactive neuronal
inclusions in frontotemporal lobar degeneration
(FTLD) with progranulin (GRN) mutation
Authors
Armstrong, Richard A. ; Cairns, Nigel J.
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Publisher
Murcia: F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Mutations of the progranulin (GRN) gene are
a major cause of familial frontotemporal lobar
degeneration with transactive response (TAR) DNAbinding
protein of 43kDa (TDP-43) proteinopathy
(FTLD-TDP). We studied the spatial patterns of TDP-43
immunoreactive neuronal cytoplasmic inclusions (NCI)
and neuronal intranuclear inclusions (NII) in histological
sections of the frontal and temporal lobe in eight cases of
FTLD-TDP with GRN mutation using morphometric
methods and spatial pattern analysis. In neocortical
regions, the NCI were clustered and the clusters were
regularly distributed parallel to the pia mater; 58% of
regions analysed exhibiting this pattern. The NII were
present in regularly distributed clusters in 35% of
regions but also randomly distributed in many areas. In
neocortical regions, the sizes of the regular clusters of
NCI and NII were 400-800 µm, approximating to the
size of the modular columns of the cortico-cortical
projections, in 31% and 36% of regions respectively.
The NCI and NII also exhibited regularly spaced
clustering in sectors CA1/2 of the hippocampus and in
the dentate gyrus. The clusters of NCI and NII were not
spatially correlated. The data suggest degeneration of the
cortico-cortical and cortico-hippocampal pathways in
FTLD-TDP with GRN mutation, the NCI and NII
affecting different clusters of neurons.
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