guest ::
| Home > Publications Database > hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations. > print |
| Home > Publications Database > hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations. > print |
| 001 | 136809 | ||
| 005 | 20250324104024.0 | ||
| 024 | 7 | _ | |a 10.1007/s00401-013-1088-7 |2 doi |
| 024 | 7 | _ | |a pmid:23381195 |2 pmid |
| 024 | 7 | _ | |a 0001-6322 |2 ISSN |
| 024 | 7 | _ | |a 1432-0533 |2 ISSN |
| 024 | 7 | _ | |a altmetric:2185996 |2 altmetric |
| 037 | _ | _ | |a DZNE-2020-03131 |
| 041 | _ | _ | |a English |
| 082 | _ | _ | |a 610 |
| 100 | 1 | _ | |a Mori, Kohji |0 P:(DE-HGF)0 |b 0 |
| 245 | _ | _ | |a hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations. |
| 260 | _ | _ | |a Heidelberg |c 2013 |b Springer |
| 264 | _ | 1 | |3 online |2 Crossref |b Springer Science and Business Media LLC |c 2013-02-05 |
| 264 | _ | 1 | |3 print |2 Crossref |b Springer Science and Business Media LLC |c 2013-03-01 |
| 336 | 7 | _ | |a article |2 DRIVER |
| 336 | 7 | _ | |a Output Types/Journal article |2 DataCite |
| 336 | 7 | _ | |a Journal Article |b journal |m journal |0 PUB:(DE-HGF)16 |s 1742809184_22034 |2 PUB:(DE-HGF) |
| 336 | 7 | _ | |a ARTICLE |2 BibTeX |
| 336 | 7 | _ | |a JOURNAL_ARTICLE |2 ORCID |
| 336 | 7 | _ | |a Journal Article |0 0 |2 EndNote |
| 520 | _ | _ | |a Genetic 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. |
| 536 | _ | _ | |a 342 - Disease Mechanisms and Model Systems (POF3-342) |0 G:(DE-HGF)POF3-342 |c POF3-342 |f POF III |x 0 |
| 536 | _ | _ | |a 344 - Clinical and Health Care Research (POF3-344) |0 G:(DE-HGF)POF3-344 |c POF3-344 |f POF III |x 1 |
| 542 | _ | _ | |i 2013-02-05 |2 Crossref |u http://www.springer.com/tdm |
| 588 | _ | _ | |a Dataset connected to CrossRef, PubMed, |
| 650 | _ | 7 | |a Adaptor Proteins, Signal Transducing |2 NLM Chemicals |
| 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 HNRNPA3 protein, human |2 NLM Chemicals |
| 650 | _ | 7 | |a Heterogeneous-Nuclear Ribonucleoprotein Group A-B |2 NLM Chemicals |
| 650 | _ | 7 | |a Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a RNA, Small Interfering |2 NLM Chemicals |
| 650 | _ | 7 | |a SQSTM1 protein, human |2 NLM Chemicals |
| 650 | _ | 7 | |a Sequestosome-1 Protein |2 NLM Chemicals |
| 650 | _ | 2 | |a Adaptor Proteins, Signal Transducing: metabolism |2 MeSH |
| 650 | _ | 2 | |a Amyotrophic Lateral Sclerosis: genetics |2 MeSH |
| 650 | _ | 2 | |a Amyotrophic Lateral Sclerosis: metabolism |2 MeSH |
| 650 | _ | 2 | |a Amyotrophic Lateral Sclerosis: pathology |2 MeSH |
| 650 | _ | 2 | |a C9orf72 Protein |2 MeSH |
| 650 | _ | 2 | |a Chromatography, High Pressure Liquid |2 MeSH |
| 650 | _ | 2 | |a DNA-Binding Proteins: metabolism |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 Gene Expression Regulation: genetics |2 MeSH |
| 650 | _ | 2 | |a HEK293 Cells |2 MeSH |
| 650 | _ | 2 | |a Heterogeneous-Nuclear Ribonucleoprotein Group A-B: 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 Mass Spectrometry |2 MeSH |
| 650 | _ | 2 | |a Mutation: genetics |2 MeSH |
| 650 | _ | 2 | |a Proteins: genetics |2 MeSH |
| 650 | _ | 2 | |a RNA, Small Interfering: metabolism |2 MeSH |
| 650 | _ | 2 | |a Repetitive Sequences, Nucleic Acid: physiology |2 MeSH |
| 650 | _ | 2 | |a Sequestosome-1 Protein |2 MeSH |
| 650 | _ | 2 | |a Transfection |2 MeSH |
| 700 | 1 | _ | |a Lammich, Sven |0 P:(DE-HGF)0 |b 1 |
| 700 | 1 | _ | |a Mackenzie, Ian R A |0 P:(DE-HGF)0 |b 2 |
| 700 | 1 | _ | |a Forné, Ignasi |0 P:(DE-HGF)0 |b 3 |
| 700 | 1 | _ | |a Zilow, Sonja |0 P:(DE-HGF)0 |b 4 |
| 700 | 1 | _ | |a Kretzschmar, Hans |0 P:(DE-HGF)0 |b 5 |
| 700 | 1 | _ | |a Edbauer, Dieter |0 P:(DE-2719)2231621 |b 6 |u dzne |
| 700 | 1 | _ | |a Janssens, Jonathan |0 P:(DE-HGF)0 |b 7 |
| 700 | 1 | _ | |a Kleinberger, Gernot |0 P:(DE-2719)9000907 |b 8 |u dzne |
| 700 | 1 | _ | |a Cruts, Marc |0 P:(DE-HGF)0 |b 9 |
| 700 | 1 | _ | |a Herms, Jochen |0 P:(DE-2719)2810441 |b 10 |u dzne |
| 700 | 1 | _ | |a Neumann, Manuela |0 P:(DE-2719)2810592 |b 11 |u dzne |
| 700 | 1 | _ | |a Van Broeckhoven, Christine |0 P:(DE-HGF)0 |b 12 |
| 700 | 1 | _ | |a Arzberger, Thomas |0 P:(DE-HGF)0 |b 13 |
| 700 | 1 | _ | |a Haass, Christian |0 P:(DE-2719)2202037 |b 14 |e Last author |u dzne |
| 773 | 1 | 8 | |a 10.1007/s00401-013-1088-7 |b : Springer Science and Business Media LLC, 2013-02-05 |n 3 |p 413-423 |3 journal-article |2 Crossref |t Acta Neuropathologica |v 125 |y 2013 |x 0001-6322 |
| 773 | _ | _ | |a 10.1007/s00401-013-1088-7 |g Vol. 125, no. 3, p. 413 - 423 |0 PERI:(DE-600)1458410-4 |n 3 |q 125:3<413 - 423 |p 413-423 |t Acta neuropathologica |v 125 |y 2013 |x 0001-6322 |
| 856 | 4 | _ | |u https://pub.dzne.de/record/136809/files/DZNE-2020-03131_Restricted.pdf |
| 856 | 4 | _ | |u https://pub.dzne.de/record/136809/files/DZNE-2020-03131_Restricted.pdf?subformat=pdfa |x pdfa |
| 909 | C | O | |p VDB |o oai:pub.dzne.de:136809 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 6 |6 P:(DE-2719)2231621 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 8 |6 P:(DE-2719)9000907 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 10 |6 P:(DE-2719)2810441 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 11 |6 P:(DE-2719)2810592 |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 14 |6 P:(DE-2719)2202037 |
| 913 | 1 | _ | |a DE-HGF |b Gesundheit |l Erkrankungen des Nervensystems |1 G:(DE-HGF)POF3-340 |0 G:(DE-HGF)POF3-342 |3 G:(DE-HGF)POF3 |2 G:(DE-HGF)POF3-300 |4 G:(DE-HGF)POF |v Disease Mechanisms and Model Systems |x 0 |
| 913 | 1 | _ | |a DE-HGF |b Gesundheit |l Erkrankungen des Nervensystems |1 G:(DE-HGF)POF3-340 |0 G:(DE-HGF)POF3-344 |3 G:(DE-HGF)POF3 |2 G:(DE-HGF)POF3-300 |4 G:(DE-HGF)POF |v Clinical and Health Care Research |x 1 |
| 914 | 1 | _ | |y 2013 |
| 915 | _ | _ | |a Nationallizenz |0 StatID:(DE-HGF)0420 |2 StatID |
| 915 | _ | _ | |a JCR |0 StatID:(DE-HGF)0100 |2 StatID |b ACTA NEUROPATHOL : 2017 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0200 |2 StatID |b SCOPUS |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0300 |2 StatID |b Medline |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0310 |2 StatID |b NCBI Molecular Biology Database |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0600 |2 StatID |b Ebsco Academic Search |
| 915 | _ | _ | |a Peer Review |0 StatID:(DE-HGF)0030 |2 StatID |b ASC |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0199 |2 StatID |b Clarivate Analytics Master Journal List |
| 915 | _ | _ | |a WoS |0 StatID:(DE-HGF)0110 |2 StatID |b Science Citation Index |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0150 |2 StatID |b Web of Science Core Collection |
| 915 | _ | _ | |a WoS |0 StatID:(DE-HGF)0111 |2 StatID |b Science Citation Index Expanded |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)1030 |2 StatID |b Current Contents - Life Sciences |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)1050 |2 StatID |b BIOSIS Previews |
| 915 | _ | _ | |a IF >= 15 |0 StatID:(DE-HGF)9915 |2 StatID |b ACTA NEUROPATHOL : 2017 |
| 920 | 1 | _ | |0 I:(DE-2719)1110004 |k AG Edbauer |l Cell Biology of Neurodegeneration |x 0 |
| 920 | 1 | _ | |0 I:(DE-2719)1110001 |k AG Herms |l Translational Brain Research |x 1 |
| 920 | 1 | _ | |0 I:(DE-2719)1210003 |k AG Neumann |l Molecular Neuropathology of Neurodegenerative Diseases |x 2 |
| 980 | _ | _ | |a journal |
| 980 | _ | _ | |a VDB |
| 980 | _ | _ | |a I:(DE-2719)1110004 |
| 980 | _ | _ | |a I:(DE-2719)1110001 |
| 980 | _ | _ | |a I:(DE-2719)1210003 |
| 980 | _ | _ | |a UNRESTRICTED |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-011-0911-2 |2 Crossref |o 10.1007/s00401-011-0911-2 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.bbrc.2006.10.093 |2 Crossref |o 10.1016/j.bbrc.2006.10.093 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nature05016 |2 Crossref |o 10.1038/nature05016 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1002/ana.21612 |2 Crossref |o 10.1002/ana.21612 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-012-0970-z |2 Crossref |o 10.1007/s00401-012-0970-z |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-012-1045-x |2 Crossref |o 10.1007/s00401-012-1045-x |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nature05017 |2 Crossref |o 10.1038/nature05017 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.neuron.2011.09.011 |2 Crossref |o 10.1016/j.neuron.2011.09.011 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1093/nar/11.5.1475 |2 Crossref |o 10.1093/nar/11.5.1475 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/emboj.2012.261 |2 Crossref |o 10.1038/emboj.2012.261 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nature09320 |2 Crossref |o 10.1038/nature09320 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/srep01016 |2 Crossref |o 10.1038/srep01016 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1002/mds.23637 |2 Crossref |o 10.1002/mds.23637 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/S1474-4422(11)70261-7 |2 Crossref |o 10.1016/S1474-4422(11)70261-7 |
| 999 | C | 5 | |y 2013 |2 Crossref |o E Gomez-Tortosa 2013 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nrm2101 |2 Crossref |o 10.1038/nrm2101 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00018-008-8532-1 |2 Crossref |o 10.1007/s00018-008-8532-1 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/S1097-2765(03)00488-X |2 Crossref |o 10.1016/S1097-2765(03)00488-X |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-012-1035-z |2 Crossref |o 10.1007/s00401-012-1035-z |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1126/science.1166066 |2 Crossref |o 10.1126/science.1166066 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.M200050200 |2 Crossref |o 10.1074/jbc.M200050200 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-011-0845-8 |2 Crossref |o 10.1007/s00401-011-0845-8 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nature08971 |2 Crossref |o 10.1038/nature08971 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-008-0477-9 |2 Crossref |o 10.1007/s00401-008-0477-9 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1126/science.1134108 |2 Crossref |o 10.1126/science.1134108 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.abb.2012.04.012 |2 Crossref |o 10.1016/j.abb.2012.04.012 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nrneurol.2012.117 |2 Crossref |o 10.1038/nrneurol.2012.117 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1146/annurev.neuro.29.051605.113014 |2 Crossref |o 10.1146/annurev.neuro.29.051605.113014 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.neuron.2011.09.010 |2 Crossref |o 10.1016/j.neuron.2011.09.010 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/362059a0 |2 Crossref |o 10.1038/362059a0 |
| 999 | C | 5 | |y 2012 |2 Crossref |o B Schreiner 2012 |
| 999 | C | 5 | |y 2000 |2 Crossref |o A Shevchenko 2000 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00401-012-1029-x |2 Crossref |o 10.1007/s00401-012-1029-x |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1093/brain/awr353 |2 Crossref |o 10.1093/brain/awr353 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/ng1609 |2 Crossref |o 10.1038/ng1609 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1126/science.1154584 |2 Crossref |o 10.1126/science.1154584 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1128/MCB.01023-06 |2 Crossref |o 10.1128/MCB.01023-06 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/ng.536 |2 Crossref |o 10.1038/ng.536 |
| 999 | C | 5 | |y 2012 |2 Crossref |o J Zee van der 2012 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1126/science.1165942 |2 Crossref |o 10.1126/science.1165942 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/ng1332 |2 Crossref |o 10.1038/ng1332 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/379466a0 |2 Crossref |o 10.1038/379466a0 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nature11280 |2 Crossref |o 10.1038/nature11280 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1073/pnas.1013343108 |2 Crossref |o 10.1073/pnas.1013343108 |
| Library | Collection | CLSMajor | CLSMinor | Language | Author |
|---|