% IMPORTANT: The following is UTF-8 encoded. This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.
@ARTICLE{Davidson:139228,
author = {Davidson, Yvonne S and Flood, Louis and Robinson, Andrew C
and Nihei, Yoshihiro and Mori, Kohji and Rollinson, Sara and
Richardson, Anna and Benson, Bridget C and Jones, Matthew
and Snowden, Julie S and Pickering-Brown, Stuart and Haass,
Christian and Lashley, Tammaryn and Mann, David M A},
title = {{H}eterogeneous ribonuclear protein {A}3 (hn{RNP} {A}3) is
present in dipeptide repeat protein containing inclusions in
{F}rontotemporal {L}obar {D}egeneration and {M}otor
{N}eurone disease associated with expansions in {C}9orf72
gene.},
journal = {Acta Neuropathologica Communications},
volume = {5},
number = {1},
issn = {2051-5960},
address = {London},
publisher = {Biomed Central},
reportid = {DZNE-2020-05550},
pages = {31},
year = {2017},
abstract = {Frontotemporal Lobar Degeneration (FTLD) encompasses
certain related neurodegenerative disorders which alter
behaviour, personality and language. Heterogeneous
ribonuclear proteins (hnRNPs) maintain RNA metabolism and
changes in their function may underpin the pathogenesis of
FTLD. Immunostaining for hnRNP A1, A2/B1 and A3 was
performed on sections of temporal cortex with hippocampus
from 61 patients with FTLD, stratified by pathological
hallmarks into FTLD-tau and FTLD-TDP type A, B and C
subtypes, and by genetics into patients with C9orf72
expansions, MAPT or GRN mutations, or those without known
mutation. Four patients with Motor Neurone Disease (MND)
with C9orf72 expansions and 10 healthy controls were also
studied. Semi-quantitative analysis assessed hnRNP staining
intensity in dentate gyrus (DG) and CA4 region of
hippocampus, and temporal cortex (Tcx) in the different
pathological and genetic groups.Immunostaining for hnRNP A1,
A2/B1 and A3 revealed no consistent changes in pattern or
amount of physiological staining across any of the
pathological or genetic groups. No immunostaining of any
inclusions resembling TDP-43 immunoreactive neuronal
cytoplasmic inclusions or dystrophic neurites, was seen in
either Tcx or DG of the hippocampus in any of the FTLD cases
investigated for hnRNP A1, A2/B1 and A3. However,
immunostaining for hnRNP A3 showed that inclusion bodies,
resembling those TDP-43 negative, p62-immunopositive
structures containing dipeptide repeat proteins (DPR) were
variably observed in hippocampus and cerebellum. The
proportion of cases showing hnRNP A3-immunoreactive DPR, and
the number of hnRNP A3-positive inclusions within cases, was
significantly greater in DG than in cells of CA4 region and
cerebellum, but the latter was significantly less in all
three regions compared to that detected by p62
immunostaining.},
keywords = {Aged / Aged, 80 and over / C9orf72 Protein: genetics /
Cerebellum: metabolism / Cerebellum: pathology / DNA Repeat
Expansion / DNA-Binding Proteins: metabolism / Female /
Frontotemporal Lobar Degeneration: genetics / Frontotemporal
Lobar Degeneration: metabolism / Frontotemporal Lobar
Degeneration: pathology / Heterogeneous Nuclear
Ribonucleoprotein A1: metabolism / Heterogeneous-Nuclear
Ribonucleoprotein Group A-B: metabolism / Hippocampus:
metabolism / Hippocampus: pathology / Humans / Inclusion
Bodies: metabolism / Inclusion Bodies: pathology /
Intercellular Signaling Peptides and Proteins: genetics /
Male / Middle Aged / Motor Neuron Disease: genetics / Motor
Neuron Disease: metabolism / Motor Neuron Disease: pathology
/ Progranulins / Temporal Lobe: metabolism / Temporal Lobe:
pathology / tau Proteins: genetics / C9orf72 Protein (NLM
Chemicals) / C9orf72 protein, human (NLM Chemicals) /
DNA-Binding Proteins (NLM Chemicals) / GRN protein, human
(NLM Chemicals) / HNRNPA3 protein, human (NLM Chemicals) /
Heterogeneous Nuclear Ribonucleoprotein A1 (NLM Chemicals) /
Heterogeneous-Nuclear Ribonucleoprotein Group A-B (NLM
Chemicals) / Intercellular Signaling Peptides and Proteins
(NLM Chemicals) / MAPT protein, human (NLM Chemicals) /
Progranulins (NLM Chemicals) / TDP-43 protein, human (NLM
Chemicals) / hnRNP A2 (NLM Chemicals) / tau Proteins (NLM
Chemicals)},
cin = {AG Haass},
ddc = {610},
cid = {I:(DE-2719)1110007},
pnm = {342 - Disease Mechanisms and Model Systems (POF3-342)},
pid = {G:(DE-HGF)POF3-342},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:28431575},
pmc = {pmc:PMC5399321},
doi = {10.1186/s40478-017-0437-5},
url = {https://pub.dzne.de/record/139228},
}