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Times Cited: 15
Times Cited: 15
Times Cited
Times Co-cited
Similarity
C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD.
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A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD.
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Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS.
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Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice.
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Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study.
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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD.
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Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
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Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits.
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Haploinsufficiency leads to neurodegeneration in C9ORF72 ALS/FTD human induced motor neurons.
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The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.
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The C9orf72 repeat expansion disrupts nucleocytoplasmic transport.
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C9orf72 suppresses systemic and neural inflammation induced by gut bacteria.
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C9orf72 is required for proper macrophage and microglial function in mice.
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C9orf72 nucleotide repeat structures initiate molecular cascades of disease.
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Loss of function of C9orf72 causes motor deficits in a zebrafish model of amyotrophic lateral sclerosis.
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C9orf72 ablation causes immune dysregulation characterized by leukocyte expansion, autoantibody production, and glomerulonephropathy in mice.
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RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia.
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Loss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune disease.
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C9orf72 deficiency promotes motor deficits of a C9ALS/FTD mouse model in a dose-dependent manner.
Qiang Shao, Chen Liang, Qing Chang, Wei Zhang, Mei Yang, Jian-Fu Chen. Acta Neuropathol Commun 2019
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C9orf72 binds SMCR8, localizes to lysosomes, and regulates mTORC1 signaling.
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C9orf72-mediated ALS and FTD: multiple pathways to disease.
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Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic.
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Deletion of C9ORF72 results in motor neuron degeneration and stress sensitivity in C. elegans.
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C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci.
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Sarah Mizielinska, Tammaryn Lashley, Frances E Norona, Emma L Clayton, Charlotte E Ridler, Pietro Fratta, Adrian M Isaacs. Acta Neuropathol 2013
20
Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations.
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C9orf72's Interaction with Rab GTPases-Modulation of Membrane Traffic and Autophagy.
Bor L Tang. Front Cell Neurosci 2016
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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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Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in C9orf72.
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C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking.
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C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins.
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The product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFs.
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TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
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The frontotemporal dementia-motor neuron disease continuum.
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The neuropathology associated with repeat expansions in the C9ORF72 gene.
Ian R A Mackenzie, Petra Frick, Manuela Neumann. Acta Neuropathol 2014
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Loss of C9orf72 Enhances Autophagic Activity via Deregulated mTOR and TFEB Signaling.
Janet Ugolino, Yon Ju Ji, Karen Conchina, Justin Chu, Raja Sekhar Nirujogi, Akhilesh Pandey, Nathan R Brady, Anne Hamacher-Brady, Jiou Wang. PLoS Genet 2016
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Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72-associated amyotrophic lateral sclerosis.
Tania F Gendron, Jeannie Chew, Jeannette N Stankowski, Lindsey R Hayes, Yong-Jie Zhang, Mercedes Prudencio, Yari Carlomagno, Lillian M Daughrity, Karen Jansen-West, Emilie A Perkerson,[...]. Sci Transl Med 2017
Tania F Gendron, Jeannie Chew, Jeannette N Stankowski, Lindsey R Hayes, Yong-Jie Zhang, Mercedes Prudencio, Yari Carlomagno, Lillian M Daughrity, Karen Jansen-West, Emilie A Perkerson,[...]. Sci Transl Med 2017
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C9ORF72-ALS/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo.
So Yoen Choi, Rodrigo Lopez-Gonzalez, Gopinath Krishnan, Hannah L Phillips, Alissa Nana Li, William W Seeley, Wei-Dong Yao, Sandra Almeida, Fen-Biao Gao. Nat Neurosci 2019
So Yoen Choi, Rodrigo Lopez-Gonzalez, Gopinath Krishnan, Hannah L Phillips, Alissa Nana Li, William W Seeley, Wei-Dong Yao, Sandra Almeida, Fen-Biao Gao. Nat Neurosci 2019
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C9orf72 loss-of-function: a trivial, stand-alone or additive mechanism in C9 ALS/FTD?
Elke Braems, Bart Swinnen, Ludo Van Den Bosch. Acta Neuropathol 2020
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Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia.
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Motor dysfunction and neurodegeneration in a C9orf72 mouse line expressing poly-PR.
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RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention.
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Christopher J Donnelly, Ping-Wu Zhang, Jacqueline T Pham, Aaron R Haeusler, Nipun A Mistry, Svetlana Vidensky, Elizabeth L Daley, Erin M Poth, Benjamin Hoover, Daniel M Fines,[...]. Neuron 2013
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Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion.
Dhruv Sareen, Jacqueline G O'Rourke, Pratap Meera, A K M G Muhammad, Sharday Grant, Megan Simpkinson, Shaughn Bell, Sharon Carmona, Loren Ornelas, Anais Sahabian,[...]. Sci Transl Med 2013
Dhruv Sareen, Jacqueline G O'Rourke, Pratap Meera, A K M G Muhammad, Sharday Grant, Megan Simpkinson, Shaughn Bell, Sharon Carmona, Loren Ornelas, Anais Sahabian,[...]. Sci Transl Med 2013
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Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers.
Ian R A Mackenzie, Petra Frick, Friedrich A Grässer, Tania F Gendron, Leonard Petrucelli, Neil R Cashman, Dieter Edbauer, Elisabeth Kremmer, Johannes Prudlo, Dirk Troost,[...]. Acta Neuropathol 2015
Ian R A Mackenzie, Petra Frick, Friedrich A Grässer, Tania F Gendron, Leonard Petrucelli, Neil R Cashman, Dieter Edbauer, Elisabeth Kremmer, Johannes Prudlo, Dirk Troost,[...]. Acta Neuropathol 2015
20
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.