000139228 001__ 139228 000139228 005__ 20240514120210.0 000139228 0247_ $$2doi$$a10.1186/s40478-017-0437-5 000139228 0247_ $$2pmid$$apmid:28431575 000139228 0247_ $$2pmc$$apmc:PMC5399321 000139228 0247_ $$2altmetric$$aaltmetric:19491201 000139228 037__ $$aDZNE-2020-05550 000139228 041__ $$aEnglish 000139228 082__ $$a610 000139228 1001_ $$0P:(DE-HGF)0$$aDavidson, Yvonne S$$b0 000139228 245__ $$aHeterogeneous ribonuclear protein A3 (hnRNP A3) is present in dipeptide repeat protein containing inclusions in Frontotemporal Lobar Degeneration and Motor Neurone disease associated with expansions in C9orf72 gene. 000139228 260__ $$aLondon$$bBiomed Central$$c2017 000139228 264_1 $$2Crossref$$3online$$bSpringer Science and Business Media LLC$$c2017-04-21 000139228 264_1 $$2Crossref$$3print$$bSpringer Science and Business Media LLC$$c2017-12-01 000139228 3367_ $$2DRIVER$$aarticle 000139228 3367_ $$2DataCite$$aOutput Types/Journal article 000139228 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1715606343_2123 000139228 3367_ $$2BibTeX$$aARTICLE 000139228 3367_ $$2ORCID$$aJOURNAL_ARTICLE 000139228 3367_ $$00$$2EndNote$$aJournal Article 000139228 520__ $$aFrontotemporal 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. 000139228 536__ $$0G:(DE-HGF)POF3-342$$a342 - Disease Mechanisms and Model Systems (POF3-342)$$cPOF3-342$$fPOF III$$x0 000139228 588__ $$aDataset connected to CrossRef, PubMed, 000139228 650_7 $$2NLM Chemicals$$aC9orf72 Protein 000139228 650_7 $$2NLM Chemicals$$aC9orf72 protein, human 000139228 650_7 $$2NLM Chemicals$$aDNA-Binding Proteins 000139228 650_7 $$2NLM Chemicals$$aGRN protein, human 000139228 650_7 $$2NLM Chemicals$$aHNRNPA3 protein, human 000139228 650_7 $$2NLM Chemicals$$aHeterogeneous Nuclear Ribonucleoprotein A1 000139228 650_7 $$2NLM Chemicals$$aHeterogeneous-Nuclear Ribonucleoprotein Group A-B 000139228 650_7 $$2NLM Chemicals$$aIntercellular Signaling Peptides and Proteins 000139228 650_7 $$2NLM Chemicals$$aMAPT protein, human 000139228 650_7 $$2NLM Chemicals$$aProgranulins 000139228 650_7 $$2NLM Chemicals$$aTDP-43 protein, human 000139228 650_7 $$2NLM Chemicals$$ahnRNP A2 000139228 650_7 $$2NLM Chemicals$$atau Proteins 000139228 650_2 $$2MeSH$$aAged 000139228 650_2 $$2MeSH$$aAged, 80 and over 000139228 650_2 $$2MeSH$$aC9orf72 Protein: genetics 000139228 650_2 $$2MeSH$$aCerebellum: metabolism 000139228 650_2 $$2MeSH$$aCerebellum: pathology 000139228 650_2 $$2MeSH$$aDNA Repeat Expansion 000139228 650_2 $$2MeSH$$aDNA-Binding Proteins: metabolism 000139228 650_2 $$2MeSH$$aFemale 000139228 650_2 $$2MeSH$$aFrontotemporal Lobar Degeneration: genetics 000139228 650_2 $$2MeSH$$aFrontotemporal Lobar Degeneration: metabolism 000139228 650_2 $$2MeSH$$aFrontotemporal Lobar Degeneration: pathology 000139228 650_2 $$2MeSH$$aHeterogeneous Nuclear Ribonucleoprotein A1: metabolism 000139228 650_2 $$2MeSH$$aHeterogeneous-Nuclear Ribonucleoprotein Group A-B: metabolism 000139228 650_2 $$2MeSH$$aHippocampus: metabolism 000139228 650_2 $$2MeSH$$aHippocampus: pathology 000139228 650_2 $$2MeSH$$aHumans 000139228 650_2 $$2MeSH$$aInclusion Bodies: metabolism 000139228 650_2 $$2MeSH$$aInclusion Bodies: pathology 000139228 650_2 $$2MeSH$$aIntercellular Signaling Peptides and Proteins: genetics 000139228 650_2 $$2MeSH$$aMale 000139228 650_2 $$2MeSH$$aMiddle Aged 000139228 650_2 $$2MeSH$$aMotor Neuron Disease: genetics 000139228 650_2 $$2MeSH$$aMotor Neuron Disease: metabolism 000139228 650_2 $$2MeSH$$aMotor Neuron Disease: pathology 000139228 650_2 $$2MeSH$$aProgranulins 000139228 650_2 $$2MeSH$$aTemporal Lobe: metabolism 000139228 650_2 $$2MeSH$$aTemporal Lobe: pathology 000139228 650_2 $$2MeSH$$atau Proteins: genetics 000139228 7001_ $$0P:(DE-HGF)0$$aFlood, Louis$$b1 000139228 7001_ $$0P:(DE-HGF)0$$aRobinson, Andrew C$$b2 000139228 7001_ $$0P:(DE-HGF)0$$aNihei, Yoshihiro$$b3 000139228 7001_ $$0P:(DE-HGF)0$$aMori, Kohji$$b4 000139228 7001_ $$0P:(DE-HGF)0$$aRollinson, Sara$$b5 000139228 7001_ $$0P:(DE-HGF)0$$aRichardson, Anna$$b6 000139228 7001_ $$0P:(DE-HGF)0$$aBenson, Bridget C$$b7 000139228 7001_ $$0P:(DE-HGF)0$$aJones, Matthew$$b8 000139228 7001_ $$0P:(DE-HGF)0$$aSnowden, Julie S$$b9 000139228 7001_ $$0P:(DE-HGF)0$$aPickering-Brown, Stuart$$b10 000139228 7001_ $$0P:(DE-2719)2202037$$aHaass, Christian$$b11$$udzne 000139228 7001_ $$0P:(DE-HGF)0$$aLashley, Tammaryn$$b12 000139228 7001_ $$0P:(DE-HGF)0$$aMann, David M A$$b13$$eCorresponding author 000139228 77318 $$2Crossref$$3journal-article$$a10.1186/s40478-017-0437-5$$b : Springer Science and Business Media LLC, 2017-04-21$$n1$$p31$$tActa Neuropathologica Communications$$v5$$x2051-5960$$y2017 000139228 773__ $$0PERI:(DE-600)2715589-4$$a10.1186/s40478-017-0437-5$$gVol. 5, no. 1, p. 31$$n1$$p31$$q5:1<31$$tActa Neuropathologica Communications$$v5$$x2051-5960$$y2017 000139228 8564_ $$uhttps://pub.dzne.de/record/139228/files/DZNE-2020-05550.pdf$$yOpenAccess 000139228 8564_ 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