Qijie Yang, Bin Jiao, Lu Shen. Front Genet 2020
Times Cited: 7
Times Cited: 7
Times Cited
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Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS.
Mariely DeJesus-Hernandez, Ian R Mackenzie, Bradley F Boeve, Adam L Boxer, Matt Baker, Nicola J Rutherford, Alexandra M Nicholson, NiCole A Finch, Heather Flynn, Jennifer Adamson,[...]. Neuron 2011
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A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD.
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C9orf72 is required for proper macrophage and microglial function in mice.
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Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death.
Xinmei Wen, Wenzhi Tan, Thomas Westergard, Karthik Krishnamurthy, Shashirekha S Markandaiah, Yingxiao Shi, Shaoyu Lin, Neil A Shneider, John Monaghan, Udai B Pandey,[...]. Neuron 2014
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Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS.
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The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains.
Erin G Conlon, Lei Lu, Aarti Sharma, Takashi Yamazaki, Timothy Tang, Neil A Shneider, James L Manley. Elife 2016
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C9orf72 arginine-rich dipeptide repeats inhibit UPF1-mediated RNA decay via translational repression.
Yu Sun, Aziz Eshov, Jeffrey Zhou, Atagun U Isiktas, Junjie U Guo. Nat Commun 2020
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Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS.
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The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.
Kohji Mori, Shih-Ming Weng, Thomas Arzberger, Stephanie May, Kristin Rentzsch, Elisabeth Kremmer, Bettina Schmid, Hans A Kretzschmar, Marc Cruts, Christine Van Broeckhoven,[...]. Science 2013
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Repeat-associated RNA structure and aberrant splicing.
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Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in C9orf72.
Qiang Zhu, Jie Jiang, Tania F Gendron, Melissa McAlonis-Downes, Lulin Jiang, Amy Taylor, Sandra Diaz Garcia, Somasish Ghosh Dastidar, Maria J Rodriguez, Patrick King,[...]. Nat Neurosci 2020
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RNA Misprocessing in C9orf72-Linked Neurodegeneration.
Holly V Barker, Michael Niblock, Youn-Bok Lee, Christopher E Shaw, Jean-Marc Gallo. Front Cell Neurosci 2017
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Disease Mechanisms of C9ORF72 Repeat Expansions.
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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD.
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C9orf72-mediated ALS and FTD: multiple pathways to disease.
Rubika Balendra, Adrian M Isaacs. Nat Rev Neurol 2018
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Translation of dipeptide repeat proteins from the C9ORF72 expanded repeat is associated with cellular stress.
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Discovery of a biomarker and lead small molecules to target r(GGGGCC)-associated defects in c9FTD/ALS.
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RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia.
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Pathogenic Mechanisms and Therapy Development for C9orf72 Amyotrophic Lateral Sclerosis/Frontotemporal Dementia.
Jie Jiang, John Ravits. Neurotherapeutics 2019
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Synthetic and natural small molecule TLR4 antagonists inhibit motoneuron death in cultures from ALS mouse model.
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Lymphocytic infiltrates in the spinal cord in amyotrophic lateral sclerosis.
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Transforming growth factor-Beta 1 (tgf-Beta 1) in patients with amyotrophic lateral sclerosis.
Joanna Iłzecka, Zbigniew Stelmasiak, Beata Dobosz. Cytokine 2002
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Microglial activation correlates with disease progression and upper motor neuron clinical symptoms in amyotrophic lateral sclerosis.
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Modulation of regulatory T cell function and stability by co-inhibitory receptors.
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The Cu, Zn Superoxide Dismutase: Not Only a Dismutase Enzyme.
Paolo Mondola, Simona Damiano, Anna Sasso, Mariarosaria Santillo. Front Physiol 2016
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Inflammatory profiles relate to survival in subtypes of amyotrophic lateral sclerosis.
Mads Nikolaj Olesen, Anna Wuolikainen, Anna Christine Nilsson, Martin Wirenfeldt, Karin Forsberg, Jonna Skov Madsen, Soeren Thue Lillevang, Ivan Brandslund, Peter Munch Andersen, Nasrin Asgari. Neurol Neuroimmunol Neuroinflamm 2020
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Cytotoxic CD8+ T lymphocytes expressing ALS-causing SOD1 mutant selectively trigger death of spinal motoneurons.
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Astrocytes: Key Regulators of Neuroinflammation.
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SOD1/Rag2 Mice with Low Copy Number of SOD1 Gene as a New Long-Living Immunodeficient Model of ALS.
M Majchrzak, K Drela, A Andrzejewska, P Rogujski, S Figurska, M Fiedorowicz, P Walczak, M Janowski, B Lukomska, L Stanaszek. Sci Rep 2019
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T lymphocytes potentiate endogenous neuroprotective inflammation in a mouse model of ALS.
Isaac M Chiu, Adam Chen, Yi Zheng, Bela Kosaras, Stefanos A Tsiftsoglou, Timothy K Vartanian, Robert H Brown, Michael C Carroll. Proc Natl Acad Sci U S A 2008
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A human-derived antibody targets misfolded SOD1 and ameliorates motor symptoms in mouse models of amyotrophic lateral sclerosis.
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Evidence of widespread cerebral microglial activation in amyotrophic lateral sclerosis: an [11C](R)-PK11195 positron emission tomography study.
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Midbrain microglia mediate a specific immunosuppressive response under inflammatory conditions.
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Co-cited is the co-citation frequency, indicating how many articles cite the article together with the query article. Similarity is the co-citation as percentage of the times cited of the query article or the article in the search results, whichever is the lowest. These numbers are calculated for the last 100 citations when articles are cited more than 100 times.