| Year |
Citation |
Score |
| 2024 |
Milioto C, Carcolé M, Giblin A, Coneys R, Attrebi O, Ahmed M, Harris SS, Lee BI, Yang M, Ellingford RA, Nirujogi RS, Biggs D, Salomonsson S, Zanovello M, de Oliveira P, ... ... Isaacs AM, et al. PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in C9orf72 ALS/FTD neurons. Nature Neuroscience. PMID 38424324 DOI: 10.1038/s41593-024-01589-4 |
0.619 |
|
| 2023 |
Rothstein JD, Baskerville V, Rapuri S, Mehlhop E, Jafar-Nejad P, Rigo F, Bennett F, Mizielinska S, Isaacs A, Coyne AN. GC targeting antisense oligonucleotides potently mitigate TDP-43 dysfunction in human C9orf72 ALS/FTD induced pluripotent stem cell derived neurons. Acta Neuropathologica. 147: 1. PMID 38019311 DOI: 10.1007/s00401-023-02652-3 |
0.389 |
|
| 2023 |
Vahsen BF, Nalluru S, Morgan GR, Farrimond L, Carroll E, Xu Y, Cramb KML, Amein B, Scaber J, Katsikoudi A, Candalija A, Carcolé M, Dafinca R, Isaacs AM, Wade-Martins R, et al. C9orf72-ALS human iPSC microglia are pro-inflammatory and toxic to co-cultured motor neurons via MMP9. Nature Communications. 14: 5898. PMID 37736756 DOI: 10.1038/s41467-023-41603-0 |
0.32 |
|
| 2023 |
Fisher EMC, Greensmith L, Malaspina A, Fratta P, Hanna MG, Schiavo G, Isaacs AM, Orrell RW, Cunningham TJ, Arozena AA. Opinion: more mouse models and more translation needed for ALS. Molecular Neurodegeneration. 18: 30. PMID 37143081 DOI: 10.1186/s13024-023-00619-2 |
0.629 |
|
| 2021 |
Licata NV, Cristofani R, Salomonsson S, Wilson KM, Kempthorne L, Vaizoglu D, D'Agostino VG, Pollini D, Loffredo R, Pancher M, Adami V, Bellosta P, Ratti A, Viero G, Quattrone A, ... Isaacs AM, et al. C9orf72 ALS/FTD dipeptide repeat protein levels are reduced by small molecules that inhibit PKA or enhance protein degradation. The Embo Journal. e105026. PMID 34791698 DOI: 10.15252/embj.2020105026 |
0.363 |
|
| 2021 |
Barton SK, Gregory JM, Selvaraj BT, McDade K, Henstridge CM, Spires-Jones TL, James OG, Mehta AR, Story D, Burr K, Magnani D, Isaacs AM, Smith C, Chandran S. Dysregulation in Subcellular Localization of Myelin Basic Protein mRNA Does Not Result in Altered Myelination in Amyotrophic Lateral Sclerosis. Frontiers in Neuroscience. 15: 705306. PMID 34539336 DOI: 10.3389/fnins.2021.705306 |
0.382 |
|
| 2021 |
Puentes F, Lombardi V, Lu CH, Yildiz O, Fratta P, Isaacs A, Bobeva Y, Wuu J, Benatar M, Malaspina A. Humoral response to neurofilaments and dipeptide repeats in ALS progression. Annals of Clinical and Translational Neurology. PMID 34318620 DOI: 10.1002/acn3.51428 |
0.644 |
|
| 2020 |
Quaegebeur A, Glaria I, Lashley T, Isaacs AM. Soluble and insoluble dipeptide repeat protein measurements in C9orf72-frontotemporal dementia brains show regional differential solubility and correlation of poly-GR with clinical severity. Acta Neuropathologica Communications. 8: 184. PMID 33168090 DOI: 10.1186/s40478-020-01036-y |
0.597 |
|
| 2020 |
Clayton EL, Isaacs AM. Progranulin and TMEM106B: when two become wan. Embo Reports. e51668. PMID 32985120 DOI: 10.15252/embr.202051668 |
0.378 |
|
| 2020 |
Humphrey J, Birsa N, Milioto C, McLaughlin M, Ule AM, Robaldo D, Eberle AB, Kräuchi R, Bentham M, Brown AL, Jarvis S, Bodo C, Garone MG, Devoy A, Soraru G, ... ... Isaacs AM, et al. FUS ALS-causative mutations impair FUS autoregulation and splicing factor networks through intron retention. Nucleic Acids Research. PMID 32479602 DOI: 10.1093/Nar/Gkaa410 |
0.798 |
|
| 2019 |
Morón-Oset J, Supèr T, Esser J, Isaacs AM, Grönke S, Partridge L. Glycine-alanine dipeptide repeats spread rapidly in a repeat length- and age-dependent manner in the fly brain. Acta Neuropathologica Communications. 7: 209. PMID 31843021 DOI: 10.1186/s40478-019-0860-x |
0.305 |
|
| 2019 |
Gittings LM, Boeynaems S, Lightwood D, Clargo A, Topia S, Nakayama L, Troakes C, Mann DMA, Gitler AD, Lashley T, Isaacs AM. Symmetric dimethylation of poly-GR correlates with disease duration in C9orf72 FTLD and ALS and reduces poly-GR phase separation and toxicity. Acta Neuropathologica. PMID 31832771 DOI: 10.1007/S00401-019-02104-X |
0.742 |
|
| 2019 |
Wilson KM, Muralidharan B, Isaacs AM. Relax, Don't RAN Translate It. Neuron. 104: 827-829. PMID 31805259 DOI: 10.1016/j.neuron.2019.11.014 |
0.42 |
|
| 2019 |
Yamada SB, Gendron TF, Niccoli T, Genuth NR, Grosely R, Shi Y, Glaria I, Kramer NJ, Nakayama L, Fang S, Dinger TJI, Thoeng A, Rocha G, Barna M, Puglisi JD, ... ... Isaacs AM, et al. RPS25 is required for efficient RAN translation of C9orf72 and other neurodegenerative disease-associated nucleotide repeats. Nature Neuroscience. PMID 31358992 DOI: 10.1038/S41593-019-0455-7 |
0.397 |
|
| 2019 |
Gittings LM, Foti SC, Benson BC, Gami-Patel P, Isaacs AM, Lashley T. Heterogeneous nuclear ribonucleoproteins R and Q accumulate in pathological inclusions in FTLD-FUS. Acta Neuropathologica Communications. 7: 18. PMID 30755280 DOI: 10.1186/s40478-019-0673-y |
0.756 |
|
| 2019 |
Moens TG, Niccoli T, Wilson KM, Atilano ML, Birsa N, Gittings LM, Holbling BV, Dyson MC, Thoeng A, Neeves J, Glaria I, Yu L, Bussmann J, Storkebaum E, Pardo M, ... ... Isaacs AM, et al. C9orf72 arginine-rich dipeptide proteins interact with ribosomal proteins in vivo to induce a toxic translational arrest that is rescued by eIF1A. Acta Neuropathologica. PMID 30604225 DOI: 10.1007/s00401-018-1946-4 |
0.762 |
|
| 2019 |
Laferrière F, Maniecka Z, Pérez-Berlanga M, Hruska-Plochan M, Gilhespy L, Hock EM, Wagner U, Afroz T, Boersema PJ, Barmettler G, Foti SC, Asi YT, Isaacs AM, Al-Amoudi A, Lewis A, et al. TDP-43 extracted from frontotemporal lobar degeneration subject brains displays distinct aggregate assemblies and neurotoxic effects reflecting disease progression rates. Nature Neuroscience. 22: 65-77. PMID 30559480 DOI: 10.1038/S41593-018-0294-Y |
0.617 |
|
| 2018 |
Clayton EL, Milioto C, Muralidharan B, Norona FE, Edgar JR, Soriano A, Jafar-Nejad P, Rigo F, Collinge J, Isaacs AM. Frontotemporal dementia causative CHMP2B impairs neuronal endolysosomal traffic-rescue by TMEM106B knockdown. Brain : a Journal of Neurology. 141: 3428-3442. PMID 30496365 DOI: 10.1093/Brain/Awy284 |
0.308 |
|
| 2018 |
Balendra R, Isaacs AM. C9orf72-mediated ALS and FTD: multiple pathways to disease. Nature Reviews. Neurology. 14: 544-558. PMID 30120348 DOI: 10.1038/s41582-018-0047-2 |
0.435 |
|
| 2018 |
Fratta P, Sivakumar P, Humphrey J, Lo K, Ricketts T, Oliveira H, Brito-Armas JM, Kalmar B, Ule A, Yu Y, Birsa N, Bodo C, Collins T, Conicella AE, Mejia Maza A, ... ... Isaacs AM, et al. Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis. The Embo Journal. PMID 29764981 DOI: 10.15252/Embj.201798684 |
0.783 |
|
| 2018 |
Fratta P, Isaacs AM. The snowball effect of RNA binding protein dysfunction in amyotrophic lateral sclerosis. Brain : a Journal of Neurology. 141: 1236-1238. PMID 29701791 DOI: 10.1093/brain/awy091 |
0.641 |
|
| 2018 |
Moens TG, Mizielinska S, Niccoli T, Mitchell JS, Thoeng A, Ridler CE, Grönke S, Esser J, Heslegrave A, Zetterberg H, Partridge L, Isaacs AM. Sense and antisense RNA are not toxic in Drosophila models of C9orf72-associated ALS/FTD. Acta Neuropathologica. PMID 29380049 DOI: 10.1007/S00401-017-1798-3 |
0.351 |
|
| 2018 |
Swinnen B, Bento-Abreu A, Gendron TF, Boeynaems S, Bogaert E, Nuyts R, Timmers M, Scheveneels W, Hersmus N, Wang J, Mizielinska S, Isaacs AM, Petrucelli L, Lemmens R, Van Damme P, et al. A zebrafish model for C9orf72 ALS reveals RNA toxicity as a pathogenic mechanism. Acta Neuropathologica. PMID 29302778 DOI: 10.1007/S00401-017-1796-5 |
0.4 |
|
| 2017 |
Simone R, Balendra R, Moens TG, Preza E, Wilson KM, Heslegrave A, Woodling NS, Niccoli T, Gilbert-Jaramillo J, Abdelkarim S, Clayton EL, Clarke M, Konrad MT, Nicoll AJ, Mitchell JS, ... ... Isaacs AM, et al. G-quadruplex-binding small molecules ameliorate C9orf72 FTD/ALS pathology in vitro and in vivo. Embo Molecular Medicine. PMID 29113975 DOI: 10.15252/Emmm.201707850 |
0.693 |
|
| 2017 |
Devoy A, Kalmar B, Stewart M, Park H, Burke B, Noy SJ, Redhead Y, Humphrey J, Lo K, Jaeger J, Mejia Maza A, Sivakumar P, Bertolin C, Soraru G, Plagnol V, ... ... Isaacs AM, et al. Humanized mutant FUS drives progressive motor neuron degeneration without aggregation in 'FUSDelta14' knockin mice. Brain : a Journal of Neurology. PMID 29053787 DOI: 10.1093/Brain/Awx248 |
0.791 |
|
| 2017 |
Hautbergue GM, Castelli LM, Ferraiuolo L, Sanchez-Martinez A, Cooper-Knock J, Higginbottom A, Lin YH, Bauer CS, Dodd JE, Myszczynska MA, Alam SM, Garneret P, Chandran JS, Karyka E, Stopford MJ, ... ... Isaacs AM, et al. SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits. Nature Communications. 8: 16063. PMID 28677678 DOI: 10.1038/Ncomms16063 |
0.392 |
|
| 2017 |
Humphrey J, Emmett W, Fratta P, Isaacs AM, Plagnol V. Quantitative analysis of cryptic splicing associated with TDP-43 depletion. Bmc Medical Genomics. 10: 38. PMID 28549443 DOI: 10.1186/S12920-017-0274-1 |
0.802 |
|
| 2017 |
Mizielinska S, Ridler CE, Balendra R, Thoeng A, Woodling NS, Grässer FA, Plagnol V, Lashley T, Partridge L, Isaacs AM. Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration. Acta Neuropathologica Communications. 5: 29. PMID 28420437 DOI: 10.1186/S40478-017-0432-X |
0.745 |
|
| 2017 |
Moens TG, Partridge L, Isaacs AM. Genetic models of C9orf72: what is toxic? Current Opinion in Genetics & Development. 44: 92-101. PMID 28364657 DOI: 10.1016/j.gde.2017.01.006 |
0.375 |
|
| 2016 |
Mizielinska S, Isaacs AM. NEURODEGENERATION. One target for amyotrophic lateral sclerosis therapy? Science (New York, N.Y.). 353: 647-8. PMID 27516584 DOI: 10.1126/Science.Aah5408 |
0.455 |
|
| 2015 |
Clayton EL, Mizielinska S, Edgar JR, Nielsen TT, Marshall S, Norona FE, Robbins M, Damirji H, Holm IE, Johannsen P, Nielsen JE, Asante EA, Collinge J, Isaacs AM. Frontotemporal dementia caused by CHMP2B mutation is characterised by neuronal lysosomal storage pathology. Acta Neuropathologica. 130: 511-23. PMID 26358247 DOI: 10.1007/S00401-015-1475-3 |
0.341 |
|
| 2015 |
Gami P, Murray C, Schottlaender L, Bettencourt C, De Pablo Fernandez E, Mudanohwo E, Mizielinska S, Polke JM, Holton JL, Isaacs AM, Houlden H, Revesz T, Lashley T. A 30-unit hexanucleotide repeat expansion in C9orf72 induces pathological lesions with dipeptide-repeat proteins and RNA foci, but not TDP-43 inclusions and clinical disease. Acta Neuropathologica. 130: 599-601. PMID 26347457 DOI: 10.1007/S00401-015-1473-5 |
0.651 |
|
| 2015 |
Simone R, Fratta P, Neidle S, Parkinson GN, Isaacs AM. G-quadruplexes: Emerging roles in neurodegenerative diseases and the non-coding transcriptome. Febs Letters. 589: 1653-68. PMID 25979174 DOI: 10.1016/J.Febslet.2015.05.003 |
0.592 |
|
| 2015 |
Rohrer JD, Isaacs AM, Mizielinska S, Mead S, Lashley T, Wray S, Sidle K, Fratta P, Orrell RW, Hardy J, Holton J, Revesz T, Rossor MN, Warren JD. C9orf72 expansions in frontotemporal dementia and amyotrophic lateral sclerosis. The Lancet. Neurology. 14: 291-301. PMID 25638642 DOI: 10.1016/S1474-4422(14)70233-9 |
0.788 |
|
| 2015 |
Fratta P, Polke JM, Newcombe J, Mizielinska S, Lashley T, Poulter M, Beck J, Preza E, Devoy A, Sidle K, Howard R, Malaspina A, Orrell RW, Clarke J, Lu CH, ... ... Isaacs AM, et al. Screening a UK amyotrophic lateral sclerosis cohort provides evidence of multiple origins of the C9orf72 expansion. Neurobiology of Aging. 36: 546.e1-7. PMID 25179228 DOI: 10.1016/J.Neurobiolaging.2014.07.037 |
0.721 |
|
| 2014 |
Mizielinska S, Grönke S, Niccoli T, Ridler CE, Clayton EL, Devoy A, Moens T, Norona FE, Woollacott IO, Pietrzyk J, Cleverley K, Nicoll AJ, Pickering-Brown S, Dols J, Cabecinha M, ... ... Isaacs AM, et al. C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins. Science (New York, N.Y.). 345: 1192-4. PMID 25103406 DOI: 10.1126/Science.1256800 |
0.68 |
|
| 2013 |
Mizielinska S, Lashley T, Norona FE, Clayton EL, Ridler CE, Fratta P, Isaacs AM. C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci. Acta Neuropathologica. 126: 845-57. PMID 24170096 DOI: 10.1007/S00401-013-1200-Z |
0.749 |
|
| 2013 |
Fratta P, Poulter M, Lashley T, Rohrer JD, Polke JM, Beck J, Ryan N, Hensman D, Mizielinska S, Waite AJ, Lai MC, Gendron TF, Petrucelli L, Fisher EM, Revesz T, ... ... Isaacs AM, et al. Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia. Acta Neuropathologica. 126: 401-9. PMID 23818065 DOI: 10.1007/S00401-013-1147-0 |
0.744 |
|
| 2013 |
Lashley T, Hardy J, Isaacs AM. RANTing about C9orf72. Neuron. 77: 597-8. PMID 23439112 DOI: 10.1016/J.Neuron.2013.02.009 |
0.706 |
|
| 2013 |
Beck J, Poulter M, Hensman D, Rohrer JD, Mahoney CJ, Adamson G, Campbell T, Uphill J, Borg A, Fratta P, Orrell RW, Malaspina A, Rowe J, Brown J, Hodges J, ... ... Isaacs AM, et al. Large C9orf72 hexanucleotide repeat expansions are seen in multiple neurodegenerative syndromes and are more frequent than expected in the UK population. American Journal of Human Genetics. 92: 345-53. PMID 23434116 DOI: 10.1016/J.Ajhg.2013.01.011 |
0.666 |
|
| 2012 |
Fratta P, Mizielinska S, Nicoll AJ, Zloh M, Fisher EM, Parkinson G, Isaacs AM. C9orf72 hexanucleotide repeat associated with amyotrophic lateral sclerosis and frontotemporal dementia forms RNA G-quadruplexes. Scientific Reports. 2: 1016. PMID 23264878 DOI: 10.1038/Srep01016 |
0.646 |
|
| 2012 |
Nielsen TT, Mizielinska S, Hasholt L, Isaacs AM, Nielsen JE. Reversal of pathology in CHMP2B-mediated frontotemporal dementia patient cells using RNA interference. The Journal of Gene Medicine. 14: 521-9. PMID 22786763 DOI: 10.1002/Jgm.2649 |
0.382 |
|
| 2011 |
Rohrer JD, Lashley T, Schott JM, Warren JE, Mead S, Isaacs AM, Beck J, Hardy J, de Silva R, Warrington E, Troakes C, Al-Sarraj S, King A, Borroni B, Clarkson MJ, et al. Clinical and neuroanatomical signatures of tissue pathology in frontotemporal lobar degeneration. Brain : a Journal of Neurology. 134: 2565-81. PMID 21908872 DOI: 10.1093/Brain/Awr198 |
0.637 |
|
| 2011 |
Lashley T, Rohrer JD, Bandopadhyay R, Fry C, Ahmed Z, Isaacs AM, Brelstaff JH, Borroni B, Warren JD, Troakes C, King A, Al-Saraj S, Newcombe J, Quinn N, Ostergaard K, et al. A comparative clinical, pathological, biochemical and genetic study of fused in sarcoma proteinopathies. Brain : a Journal of Neurology. 134: 2548-64. PMID 21752791 DOI: 10.1093/Brain/Awr160 |
0.617 |
|
| 2011 |
Mackenzie IR, Neumann M, Cairns NJ, Munoz DG, Isaacs AM. Novel types of frontotemporal lobar degeneration: beyond tau and TDP-43. Journal of Molecular Neuroscience : Mn. 45: 402-8. PMID 21603977 DOI: 10.1007/S12031-011-9551-1 |
0.348 |
|
| 2011 |
Rohrer JD, Lashley T, Holton J, Revesz T, Urwin H, Isaacs AM, Fox NC, Rossor MN, Warren J. The clinical and neuroanatomical phenotype of FUS associated frontotemporal lobar degeneration. Journal of Neurology, Neurosurgery, and Psychiatry. 82: 1405-7. PMID 20639383 DOI: 10.1136/Jnnp.2010.214437 |
0.599 |
|
| 2010 |
Urwin H, Authier A, Nielsen JE, Metcalf D, Powell C, Froud K, Malcolm DS, Holm I, Johannsen P, Brown J, Fisher EM, van der Zee J, Bruyland M, Van Broeckhoven C, ... ... Isaacs AM, et al. Disruption of endocytic trafficking in frontotemporal dementia with CHMP2B mutations. Human Molecular Genetics. 19: 2228-38. PMID 20223751 DOI: 10.1093/Hmg/Ddq100 |
0.314 |
|
| 2008 |
Banks GT, Kuta A, Isaacs AM, Fisher EMC. TDP-43 is a culprit in human neurodegeneration, and not just an innocent bystander Mammalian Genome. 19: 299-305. PMID 18592312 DOI: 10.1007/S00335-008-9117-X |
0.312 |
|
| 2007 |
Filimonenko M, Stuffers S, Raiborg C, Yamamoto A, Malerød L, Fisher EM, Isaacs A, Brech A, Stenmark H, Simonsen A. Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease. The Journal of Cell Biology. 179: 485-500. PMID 17984323 DOI: 10.1083/Jcb.200702115 |
0.409 |
|
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