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000138773 0247_ $$2doi$$a10.15252/embr.201541724
000138773 0247_ $$2pmid$$apmid:27461252
000138773 0247_ $$2pmc$$apmc:PMC5007570
000138773 0247_ $$2ISSN$$a1469-221X
000138773 0247_ $$2ISSN$$a1469-3178
000138773 0247_ $$2altmetric$$aaltmetric:10091664
000138773 037__ $$aDZNE-2020-05095
000138773 041__ $$aEnglish
000138773 082__ $$a570
000138773 1001_ $$0P:(DE-HGF)0$$aMori, Kohji$$b0$$eCorresponding author
000138773 245__ $$aReduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition.
000138773 260__ $$aHoboken, NJ [u.a.]$$bWiley$$c2016
000138773 264_1 $$2Crossref$$3online$$bEMBO$$c2016-07-26
000138773 264_1 $$2Crossref$$3print$$bEMBO$$c2016-09-01
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000138773 3367_ $$2ORCID$$aJOURNAL_ARTICLE
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000138773 520__ $$aIntronic hexanucleotide (G4C2) repeat expansions in C9orf72 are genetically associated with frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). The repeat RNA accumulates within RNA foci but is also translated into disease characterizing dipeptide repeat proteins (DPR). Repeat-dependent toxicity may affect nuclear import. hnRNPA3 is a heterogeneous nuclear ribonucleoprotein, which specifically binds to the G4C2 repeat RNA We now report that a reduction of nuclear hnRNPA3 leads to an increase of the repeat RNA as well as DPR production and deposition in primary neurons and a novel tissue culture model that reproduces features of the C9orf72 pathology. In fibroblasts derived from patients carrying extended C9orf72 repeats, nuclear RNA foci accumulated upon reduction of hnRNPA3. Neurons in the hippocampus of C9orf72 patients are frequently devoid of hnRNPA3. Reduced nuclear hnRNPA3 in the hippocampus of patients with extended C9orf72 repeats correlates with increased DPR deposition. Thus, reduced hnRNPA3 expression in C9orf72 cases leads to increased levels of the repeat RNA as well as enhanced production and deposition of DPR proteins and RNA foci.
000138773 536__ $$0G:(DE-HGF)POF3-344$$a344 - Clinical and Health Care Research (POF3-344)$$cPOF3-344$$fPOF III$$x0
000138773 536__ $$0G:(DE-HGF)POF3-342$$a342 - Disease Mechanisms and Model Systems (POF3-342)$$cPOF3-342$$fPOF III$$x1
000138773 542__ $$2Crossref$$i2016-07-26$$uhttp://onlinelibrary.wiley.com/termsAndConditions#vor
000138773 542__ $$2Crossref$$i2016-07-26$$uhttp://doi.wiley.com/10.1002/tdm_license_1.1
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000138773 650_7 $$2NLM Chemicals$$aC9orf72 Protein
000138773 650_7 $$2NLM Chemicals$$aC9orf72 protein, human
000138773 650_7 $$2NLM Chemicals$$aDipeptides
000138773 650_7 $$2NLM Chemicals$$aHNRNPA3 protein, human
000138773 650_7 $$2NLM Chemicals$$aHeterogeneous-Nuclear Ribonucleoprotein Group A-B
000138773 650_7 $$2NLM Chemicals$$aProteins
000138773 650_7 $$2NLM Chemicals$$aRNA, Messenger
000138773 650_7 $$2NLM Chemicals$$aRNA, Small Interfering
000138773 650_2 $$2MeSH$$aAnimals
000138773 650_2 $$2MeSH$$aBrain: metabolism
000138773 650_2 $$2MeSH$$aC9orf72 Protein
000138773 650_2 $$2MeSH$$aDipeptides: metabolism
000138773 650_2 $$2MeSH$$aFibroblasts
000138773 650_2 $$2MeSH$$aGene Expression Regulation
000138773 650_2 $$2MeSH$$aGene Knockdown Techniques
000138773 650_2 $$2MeSH$$aHeLa Cells
000138773 650_2 $$2MeSH$$aHeterogeneous-Nuclear Ribonucleoprotein Group A-B: metabolism
000138773 650_2 $$2MeSH$$aHumans
000138773 650_2 $$2MeSH$$aNeurons: metabolism
000138773 650_2 $$2MeSH$$aProtein Binding
000138773 650_2 $$2MeSH$$aProtein Transport
000138773 650_2 $$2MeSH$$aProteins: genetics
000138773 650_2 $$2MeSH$$aPyramidal Cells: metabolism
000138773 650_2 $$2MeSH$$aRNA Transport
000138773 650_2 $$2MeSH$$aRNA, Messenger: genetics
000138773 650_2 $$2MeSH$$aRNA, Small Interfering: genetics
000138773 650_2 $$2MeSH$$aRats
000138773 7001_ $$0P:(DE-HGF)0$$aNihei, Yoshihiro$$b1
000138773 7001_ $$0P:(DE-2719)2811333$$aArzberger, Thomas$$b2
000138773 7001_ $$0P:(DE-2719)2811347$$aZhou, Qihui$$b3
000138773 7001_ $$0P:(DE-HGF)0$$aMackenzie, Ian R$$b4
000138773 7001_ $$0P:(DE-2719)2811732$$aHermann, Andreas$$b5
000138773 7001_ $$aHanisch, Frank$$b6
000138773 7001_ $$aDegeneration, German Consortium for Frontotemporal Lobar$$b7
000138773 7001_ $$aAlliance, Bavarian Brain Banking$$b8
000138773 7001_ $$0P:(DE-HGF)0$$aKamp, Frits$$b9
000138773 7001_ $$0P:(DE-HGF)0$$aNuscher, Brigitte$$b10
000138773 7001_ $$0P:(DE-2719)9000242$$aOrozco, Denise$$b11
000138773 7001_ $$0P:(DE-2719)2231621$$aEdbauer, Dieter$$b12
000138773 7001_ $$0P:(DE-2719)2202037$$aHaass, Christian$$b13$$eLast author
000138773 77318 $$2Crossref$$3journal-article$$a10.15252/embr.201541724$$b : EMBO, 2016-07-26$$n9$$p1314-1325$$tEMBO reports$$v17$$x1469-221X$$y2016
000138773 773__ $$0PERI:(DE-600)2025376-X$$a10.15252/embr.201541724$$gVol. 17, no. 9, p. 1314 - 1325$$n9$$p1314-1325$$q17:9<1314 - 1325$$tEMBO reports$$v17$$x1469-221X$$y2016
000138773 8567_ $$2Pubmed Central$$uhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC5007570
000138773 8564_ $$uhttps://pub.dzne.de/record/138773/files/DZNE-2020-05095_Restricted.pdf
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000138773 9141_ $$y2016
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000138773 9201_ $$0I:(DE-2719)1111016$$kAG Levin$$lClinical Neurodegeneration$$x0
000138773 9201_ $$0I:(DE-2719)1110004$$kAG Edbauer$$lCell Biology of Neurodegeneration$$x1
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