Home > Publications Database > Heterogeneous ribonuclear protein A3 (hnRNP A3) is present in dipeptide repeat protein containing inclusions in Frontotemporal Lobar Degeneration and Motor Neurone disease associated with expansions in C9orf72 gene. > print

001     139228
005     20240514120210.0
024 7 _ |a 10.1186/s40478-017-0437-5
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024 7 _ |a pmc:PMC5399321
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037 _ _ |a DZNE-2020-05550
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Davidson, Yvonne S
|0 P:(DE-HGF)0
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245 _ _ |a Heterogeneous ribonuclear protein A3 (hnRNP A3) is present in dipeptide repeat protein containing inclusions in Frontotemporal Lobar Degeneration and Motor Neurone disease associated with expansions in C9orf72 gene.
260 _ _ |a London
|c 2017
|b Biomed Central
264 _ 1 |3 online
|2 Crossref
|b Springer Science and Business Media LLC
|c 2017-04-21
264 _ 1 |3 print
|2 Crossref
|b Springer Science and Business Media LLC
|c 2017-12-01
336 7 _ |a article
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336 7 _ |a Output Types/Journal article
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336 7 _ |a Journal Article
|b journal
|m journal
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|s 1715606343_2123
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336 7 _ |a ARTICLE
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336 7 _ |a Journal Article
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520 _ _ |a 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.
536 _ _ |a 342 - Disease Mechanisms and Model Systems (POF3-342)
|0 G:(DE-HGF)POF3-342
|c POF3-342
|f POF III
|x 0
588 _ _ |a Dataset connected to CrossRef, PubMed,
650 _ 7 |a C9orf72 Protein
|2 NLM Chemicals
650 _ 7 |a C9orf72 protein, human
|2 NLM Chemicals
650 _ 7 |a DNA-Binding Proteins
|2 NLM Chemicals
650 _ 7 |a GRN protein, human
|2 NLM Chemicals
650 _ 7 |a HNRNPA3 protein, human
|2 NLM Chemicals
650 _ 7 |a Heterogeneous Nuclear Ribonucleoprotein A1
|2 NLM Chemicals
650 _ 7 |a Heterogeneous-Nuclear Ribonucleoprotein Group A-B
|2 NLM Chemicals
650 _ 7 |a Intercellular Signaling Peptides and Proteins
|2 NLM Chemicals
650 _ 7 |a MAPT protein, human
|2 NLM Chemicals
650 _ 7 |a Progranulins
|2 NLM Chemicals
650 _ 7 |a TDP-43 protein, human
|2 NLM Chemicals
650 _ 7 |a hnRNP A2
|2 NLM Chemicals
650 _ 7 |a tau Proteins
|2 NLM Chemicals
650 _ 2 |a Aged
|2 MeSH
650 _ 2 |a Aged, 80 and over
|2 MeSH
650 _ 2 |a C9orf72 Protein: genetics
|2 MeSH
650 _ 2 |a Cerebellum: metabolism
|2 MeSH
650 _ 2 |a Cerebellum: pathology
|2 MeSH
650 _ 2 |a DNA Repeat Expansion
|2 MeSH
650 _ 2 |a DNA-Binding Proteins: metabolism
|2 MeSH
650 _ 2 |a Female
|2 MeSH
650 _ 2 |a Frontotemporal Lobar Degeneration: genetics
|2 MeSH
650 _ 2 |a Frontotemporal Lobar Degeneration: metabolism
|2 MeSH
650 _ 2 |a Frontotemporal Lobar Degeneration: pathology
|2 MeSH
650 _ 2 |a Heterogeneous Nuclear Ribonucleoprotein A1: metabolism
|2 MeSH
650 _ 2 |a Heterogeneous-Nuclear Ribonucleoprotein Group A-B: metabolism
|2 MeSH
650 _ 2 |a Hippocampus: metabolism
|2 MeSH
650 _ 2 |a Hippocampus: pathology
|2 MeSH
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Inclusion Bodies: metabolism
|2 MeSH
650 _ 2 |a Inclusion Bodies: pathology
|2 MeSH
650 _ 2 |a Intercellular Signaling Peptides and Proteins: genetics
|2 MeSH
650 _ 2 |a Male
|2 MeSH
650 _ 2 |a Middle Aged
|2 MeSH
650 _ 2 |a Motor Neuron Disease: genetics
|2 MeSH
650 _ 2 |a Motor Neuron Disease: metabolism
|2 MeSH
650 _ 2 |a Motor Neuron Disease: pathology
|2 MeSH
650 _ 2 |a Progranulins
|2 MeSH
650 _ 2 |a Temporal Lobe: metabolism
|2 MeSH
650 _ 2 |a Temporal Lobe: pathology
|2 MeSH
650 _ 2 |a tau Proteins: genetics
|2 MeSH
700 1 _ |a Flood, Louis
|0 P:(DE-HGF)0
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700 1 _ |a Robinson, Andrew C
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700 1 _ |a Nihei, Yoshihiro
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700 1 _ |a Mori, Kohji
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700 1 _ |a Rollinson, Sara
|0 P:(DE-HGF)0
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700 1 _ |a Richardson, Anna
|0 P:(DE-HGF)0
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700 1 _ |a Benson, Bridget C
|0 P:(DE-HGF)0
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700 1 _ |a Jones, Matthew
|0 P:(DE-HGF)0
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700 1 _ |a Snowden, Julie S
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700 1 _ |a Pickering-Brown, Stuart
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700 1 _ |a Haass, Christian
|0 P:(DE-2719)2202037
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700 1 _ |a Lashley, Tammaryn
|0 P:(DE-HGF)0
|b 12
700 1 _ |a Mann, David M A
|0 P:(DE-HGF)0
|b 13
|e Corresponding author
773 1 8 |a 10.1186/s40478-017-0437-5
|b : Springer Science and Business Media LLC, 2017-04-21
|n 1
|p 31
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|t Acta Neuropathologica Communications
|v 5
|y 2017
|x 2051-5960
773 _ _ |a 10.1186/s40478-017-0437-5
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910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
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