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000136809 0247_ $$2doi$$a10.1007/s00401-013-1088-7
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000136809 037__ $$aDZNE-2020-03131
000136809 041__ $$aEnglish
000136809 082__ $$a610
000136809 1001_ $$0P:(DE-HGF)0$$aMori, Kohji$$b0
000136809 245__ $$ahnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations.
000136809 260__ $$aHeidelberg$$bSpringer$$c2013
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000136809 520__ $$aGenetic analysis revealed the hexanucleotide repeat expansion GGGGCC within the regulatory region of the gene C9orf72 as the most common cause of familial amyotrophic lateral sclerosis and the second most common cause of frontotemporal lobar degeneration. Since repeat expansions might cause RNA toxicity via sequestration of RNA-binding proteins, we searched for proteins capable of binding to GGGGCC repeats. In vitro-transcribed biotinylated RNA containing hexanucleotide GGGGCC or, as control, AAAACC repeats were incubated with nuclear protein extracts. Using stringent filtering protocols 20 RNA-binding proteins with a variety of different functions in RNA metabolism, translation and transport were identified. A subset of these proteins was further investigated by immunohistochemistry in human autopsy brains. This revealed that hnRNP A3 formed neuronal cytoplasmic and intranuclear inclusions in the hippocampus of patients with C9orf72 repeat extensions. Confocal microcopy showed that these inclusions belong to the group of the so far enigmatic p62-positive/TDP-43 negative inclusions characteristically seen in autopsy cases of diseased C9orf72 repeat expansion carriers. Thus, we have identified one protein component of these pathognomonic inclusions.
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000136809 536__ $$0G:(DE-HGF)POF3-344$$a344 - Clinical and Health Care Research (POF3-344)$$cPOF3-344$$fPOF III$$x1
000136809 542__ $$2Crossref$$i2013-02-05$$uhttp://www.springer.com/tdm
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000136809 650_7 $$2NLM Chemicals$$aAdaptor Proteins, Signal Transducing
000136809 650_7 $$2NLM Chemicals$$aC9orf72 Protein
000136809 650_7 $$2NLM Chemicals$$aC9orf72 protein, human
000136809 650_7 $$2NLM Chemicals$$aDNA-Binding Proteins
000136809 650_7 $$2NLM Chemicals$$aHNRNPA3 protein, human
000136809 650_7 $$2NLM Chemicals$$aHeterogeneous-Nuclear Ribonucleoprotein Group A-B
000136809 650_7 $$2NLM Chemicals$$aProteins
000136809 650_7 $$2NLM Chemicals$$aRNA, Small Interfering
000136809 650_7 $$2NLM Chemicals$$aSQSTM1 protein, human
000136809 650_7 $$2NLM Chemicals$$aSequestosome-1 Protein
000136809 650_2 $$2MeSH$$aAdaptor Proteins, Signal Transducing: metabolism
000136809 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: genetics
000136809 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: metabolism
000136809 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: pathology
000136809 650_2 $$2MeSH$$aC9orf72 Protein
000136809 650_2 $$2MeSH$$aChromatography, High Pressure Liquid
000136809 650_2 $$2MeSH$$aDNA-Binding Proteins: metabolism
000136809 650_2 $$2MeSH$$aFrontotemporal Lobar Degeneration: genetics
000136809 650_2 $$2MeSH$$aFrontotemporal Lobar Degeneration: metabolism
000136809 650_2 $$2MeSH$$aFrontotemporal Lobar Degeneration: pathology
000136809 650_2 $$2MeSH$$aGene Expression Regulation: genetics
000136809 650_2 $$2MeSH$$aHEK293 Cells
000136809 650_2 $$2MeSH$$aHeterogeneous-Nuclear Ribonucleoprotein Group A-B: metabolism
000136809 650_2 $$2MeSH$$aHippocampus: pathology
000136809 650_2 $$2MeSH$$aHumans
000136809 650_2 $$2MeSH$$aInclusion Bodies: metabolism
000136809 650_2 $$2MeSH$$aInclusion Bodies: pathology
000136809 650_2 $$2MeSH$$aMass Spectrometry
000136809 650_2 $$2MeSH$$aMutation: genetics
000136809 650_2 $$2MeSH$$aProteins: genetics
000136809 650_2 $$2MeSH$$aRNA, Small Interfering: metabolism
000136809 650_2 $$2MeSH$$aRepetitive Sequences, Nucleic Acid: physiology
000136809 650_2 $$2MeSH$$aSequestosome-1 Protein
000136809 650_2 $$2MeSH$$aTransfection
000136809 7001_ $$0P:(DE-HGF)0$$aLammich, Sven$$b1
000136809 7001_ $$0P:(DE-HGF)0$$aMackenzie, Ian R A$$b2
000136809 7001_ $$0P:(DE-HGF)0$$aForné, Ignasi$$b3
000136809 7001_ $$0P:(DE-HGF)0$$aZilow, Sonja$$b4
000136809 7001_ $$0P:(DE-HGF)0$$aKretzschmar, Hans$$b5
000136809 7001_ $$0P:(DE-2719)2231621$$aEdbauer, Dieter$$b6$$udzne
000136809 7001_ $$0P:(DE-HGF)0$$aJanssens, Jonathan$$b7
000136809 7001_ $$0P:(DE-2719)9000907$$aKleinberger, Gernot$$b8$$udzne
000136809 7001_ $$0P:(DE-HGF)0$$aCruts, Marc$$b9
000136809 7001_ $$0P:(DE-2719)2810441$$aHerms, Jochen$$b10$$udzne
000136809 7001_ $$0P:(DE-2719)2810592$$aNeumann, Manuela$$b11$$udzne
000136809 7001_ $$0P:(DE-HGF)0$$aVan Broeckhoven, Christine$$b12
000136809 7001_ $$0P:(DE-HGF)0$$aArzberger, Thomas$$b13
000136809 7001_ $$0P:(DE-2719)2202037$$aHaass, Christian$$b14$$eLast author$$udzne
000136809 77318 $$2Crossref$$3journal-article$$a10.1007/s00401-013-1088-7$$b : Springer Science and Business Media LLC, 2013-02-05$$n3$$p413-423$$tActa Neuropathologica$$v125$$x0001-6322$$y2013
000136809 773__ $$0PERI:(DE-600)1458410-4$$a10.1007/s00401-013-1088-7$$gVol. 125, no. 3, p. 413 - 423$$n3$$p413-423$$q125:3<413 - 423$$tActa neuropathologica$$v125$$x0001-6322$$y2013
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