Works by Isaacs, Adrian


Results: 72
    1

    Regulation of Postsynaptic Function by the Dementia-Related ESCRT-III Subunit CHMP2B.

    Published in:
    Journal of Neuroscience, 2015, v. 35, n. 7, p. 3155, doi. 10.1523/JNEUROSCI.0586-14.2015
    By:
    • Chassefeyre, Romain;
    • Martínez-Hernández, José;
    • Bertaso, Federica;
    • Bouquier, Nathalie;
    • Blot, Béatrice;
    • Laporte, Marine;
    • Fraboulet, Sandrine;
    • Couté, Yohann;
    • Devoy, Anny;
    • Isaacs, Adrian M.;
    • Pernet-Gallay, Karin;
    • Sadoul, Rémy;
    • Fagni, Laurent;
    • Goldberg, Yves
    Publication type:
    Article
    2

    Transcriptome-wide RNA binding analysis of C9orf72 poly(PR) dipeptides.

    Published in:
    Life Science Alliance, 2023, v. 6, n. 9, p. 1, doi. 10.26508/lsa.202201824
    By:
    • Balendra, Rubika;
    • de los Mozos, Igor Ruiz;
    • Odeh, Hana M.;
    • Glaria, Idoia;
    • Milioto, Carmelo;
    • Wilson, Katherine M.;
    • Ule, Agnieszka M.;
    • Hallegger, Martina;
    • Masino, Laura;
    • Martin, Stephen;
    • Patani, Rickie;
    • Shorter, James;
    • Ule, Jernej;
    • Isaacs, Adrian M.
    Publication type:
    Article
    3
    4
    5

    A Mutation in Af4 Is Predicted to Cause Cerebellar Ataxia and Cataracts in the Robotic Mouse.

    Published in:
    Journal of Neuroscience, 2003, v. 23, n. 5, p. 1631, doi. 10.1523/JNEUROSCI.23-05-01631.2003
    By:
    • Isaacs, Adrian M.;
    • Oliver, Peter L.;
    • Jones, Emma L.;
    • Jeans, Alexander;
    • Potter, Allyson;
    • Hovik, Berit H.;
    • Nolan, Patrick M.;
    • Vizor, Lucie;
    • Glenister, Peter;
    • Simon, A. Katharina;
    • Gray, Ian C.;
    • Spurr, Nigel H.;
    • Brown, Steve D.M.;
    • Hunter, A. Jackie;
    • Davies, Kay E.
    Publication type:
    Article
    6

    Quantitative Assessment of Eye Phenotypes for Functional Genetic Studies Using Drosophila melanogaster.

    Published in:
    G3: Genes | Genomes | Genetics, 2016, v. 6, n. 5, p. 1427, doi. 10.1534/g3.116.027060
    By:
    • Iyer, Janani;
    • Wang, Qingyu;
    • Le, Thanh;
    • Pizzo, Lucilla;
    • Grönke, Sebastian;
    • Ambegaokar, Surendra S.;
    • Imai, Yuzuru;
    • Srivastava, Ashutosh;
    • Troisí, Beatriz Llamusí;
    • Mardon, Graeme;
    • Artero, Ruben;
    • Jackson, George R.;
    • Isaacs, Adrian M.;
    • Partridge, Linda;
    • Bingwei Lu;
    • Kumar, Justin P.;
    • Girirajan, Santhosh
    Publication type:
    Article
    7

    Six generations of CHMP2B‐mediated Frontotemporal Dementia: Clinical features, predictive testing, progression, and survival.

    Published in:
    Acta Neurologica Scandinavica, 2022, v. 145, n. 5, p. 529, doi. 10.1111/ane.13578
    By:
    • Roos, Peter;
    • Johannsen, Peter;
    • Lindquist, Suzanne G.;
    • Brown, Jeremy M.;
    • Waldemar, Gunhild;
    • Duno, Morten;
    • Nielsen, Troels T.;
    • Budtz‐Jørgensen, Esben;
    • Gydesen, Susanne;
    • Holm, Ida E.;
    • Collinge, John;
    • Isaacs, Adrian M.;
    • Nielsen, Jørgen E.;
    • Gade, Anders;
    • Stokholm, Jette;
    • Thusgaard, Tove;
    • Fisher, Elizabeth M.C.;
    • Englund, Elisabet
    Publication type:
    Article
    8
    9
    10
    11
    12
    13
    14
    15

    Localization of frontotemporal dementia with parkinsonism in an Australian kindred to chromosome 17q21-22.

    Published in:
    Annals of Neurology, 1997, v. 42, n. 5, p. 794, doi. 10.1002/ana.410420516
    By:
    • Baker, Matt;
    • Kwok, John B. J.;
    • Kucera, Steve;
    • Crook, Richard;
    • Farrer, Matthew;
    • Houlden, Henry;
    • Isaacs, Adrian;
    • Lincoln, Sarah;
    • Onstead, Luisa;
    • Hardy, John;
    • Wittenberg, Leonie;
    • Dodd, Peter;
    • Webb, Sonja;
    • Hayward, Nick;
    • Tannenberg, Tony;
    • Andreadis, Athena;
    • Hallupp, Marianne;
    • Schofield, Peter;
    • Dark, Frances;
    • Hutton, Michael
    Publication type:
    Article
    16

    C9orf72 ALS/FTD dipeptide repeat protein levels are reduced by small molecules that inhibit PKA or enhance protein degradation.

    Published in:
    EMBO Journal, 2022, v. 41, n. 1, p. 1, doi. 10.15252/embj.2020105026
    By:
    • Licata, Nausicaa V;
    • Cristofani, Riccardo;
    • Salomonsson, Sally;
    • Wilson, Katherine M;
    • Kempthorne, Liam;
    • Vaizoglu, Deniz;
    • D'Agostino, Vito G;
    • Pollini, Daniele;
    • Loffredo, Rosa;
    • Pancher, Michael;
    • Adami, Valentina;
    • Bellosta, Paola;
    • Ratti, Antonia;
    • Viero, Gabriella;
    • Quattrone, Alessandro;
    • Isaacs, Adrian M;
    • Poletti, Angelo;
    • Provenzani, Alessandro
    Publication type:
    Article
    17

    Molecular pathology, developmental changes and synaptic dysfunction in (pre-) symptomatic human C9ORF72-ALS/FTD cerebral organoids.

    Published in:
    Acta Neuropathologica Communications, 2024, p. 1, doi. 10.1186/s40478-024-01857-1
    By:
    • van der Geest, Astrid T.;
    • Jakobs, Channa E.;
    • Ljubikj, Tijana;
    • Huffels, Christiaan F. M.;
    • Cañizares Luna, Marta;
    • Vieira de Sá, Renata;
    • Adolfs, Youri;
    • de Wit, Marina;
    • Rutten, Daan H.;
    • Kaal, Marthe;
    • Zwartkruis, Maria M.;
    • Carcolé, Mireia;
    • Groen, Ewout J. N.;
    • Hol, Elly M.;
    • Basak, Onur;
    • Isaacs, Adrian M.;
    • Westeneng, Henk-Jan;
    • van den Berg, Leonard H.;
    • Veldink, Jan H.;
    • Schlegel, Domino K.
    Publication type:
    Article
    18
    19
    20
    21
    22
    23
    24

    SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits.

    Published in:
    Nature Communications, 2017, v. 8, n. 7, p. 16063, doi. 10.1038/ncomms16063
    By:
    • Hautbergue, Guillaume M.;
    • Castelli, Lydia M.;
    • Ferraiuolo, Laura;
    • Sanchez-Martinez, Alvaro;
    • Cooper-Knock, Johnathan;
    • Higginbottom, Adrian;
    • Lin, Ya-Hui;
    • Bauer, Claudia S.;
    • Dodd, Jennifer E.;
    • Myszczynska, Monika A.;
    • Alam, Sarah M.;
    • Garneret, Pierre;
    • Chandran, Jayanth S.;
    • Karyka, Evangelia;
    • Stopford, Matthew J.;
    • Smith, Emma F.;
    • Kirby, Janine;
    • Meyer, Kathrin;
    • Kaspar, Brian K.;
    • Isaacs, Adrian M.
    Publication type:
    Article
    25
    26
    27

    C9orf72 arginine-rich dipeptide proteins interact with ribosomal proteins in vivo to induce a toxic translational arrest that is rescued by eIF1A.

    Published in:
    Acta Neuropathologica, 2019, v. 137, n. 3, p. 487, doi. 10.1007/s00401-018-1946-4
    By:
    • Moens, Thomas G.;
    • Niccoli, Teresa;
    • Wilson, Katherine M.;
    • Atilano, Magda L.;
    • Birsa, Nicol;
    • Gittings, Lauren M.;
    • Holbling, Benedikt V.;
    • Dyson, Miranda C.;
    • Thoeng, Annora;
    • Neeves, Jacob;
    • Glaria, Idoia;
    • Yu, Lu;
    • Bussmann, Julia;
    • Storkebaum, Erik;
    • Pardo, Mercedes;
    • Choudhary, Jyoti S.;
    • Fratta, Pietro;
    • Partridge, Linda;
    • Isaacs, Adrian M.
    Publication type:
    Article
    28
    29

    A zebrafish model for C9orf72 ALS reveals RNA toxicity as a pathogenic mechanism.

    Published in:
    Acta Neuropathologica, 2018, v. 135, n. 3, p. 427, doi. 10.1007/s00401-017-1796-5
    By:
    • Swinnen, Bart;
    • Bento-Abreu, Andre;
    • Gendron, Tania F.;
    • Boeynaems, Steven;
    • Bogaert, Elke;
    • Nuyts, Rik;
    • Timmers, Mieke;
    • Scheveneels, Wendy;
    • Hersmus, Nicole;
    • Wang, Jiou;
    • Mizielinska, Sarah;
    • Isaacs, Adrian M.;
    • Petrucelli, Leonard;
    • Lemmens, Robin;
    • Van Damme, Philip;
    • Van Den Bosch, Ludo;
    • Robberecht, Wim
    Publication type:
    Article
    30
    31
    32
    33

    Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia.

    Published in:
    Acta Neuropathologica, 2013, v. 126, n. 3, p. 401, doi. 10.1007/s00401-013-1147-0
    By:
    • Fratta, Pietro;
    • Poulter, Mark;
    • Lashley, Tammaryn;
    • Rohrer, Jonathan;
    • Polke, James;
    • Beck, Jon;
    • Ryan, Natalie;
    • Hensman, Davina;
    • Mizielinska, Sarah;
    • Waite, Adrian;
    • Lai, Mang-Ching;
    • Gendron, Tania;
    • Petrucelli, Leonard;
    • Fisher, Elizabeth;
    • Revesz, Tamas;
    • Warren, Jason;
    • Collinge, John;
    • Isaacs, Adrian;
    • Mead, Simon
    Publication type:
    Article
    34
    35
    36
    37
    38
    39
    40

    Clinical and neuroanatomical signatures of tissue pathology in frontotemporal lobar degeneration.

    Published in:
    Brain: A Journal of Neurology, 2011, v. 134, n. 9, p. 2565, doi. 10.1093/brain/awr198
    By:
    • Rohrer, Jonathan D.;
    • Lashley, Tammaryn;
    • Schott, Jonathan M.;
    • Warren, Jane E.;
    • Mead, Simon;
    • Isaacs, Adrian M.;
    • Beck, Jonathan;
    • Hardy, John;
    • de Silva, Rohan;
    • Warrington, Elizabeth;
    • Troakes, Claire;
    • Al-Sarraj, Safa;
    • King, Andrew;
    • Borroni, Barbara;
    • Clarkson, Matthew J.;
    • Ourselin, Sebastien;
    • Holton, Janice L.;
    • Fox, Nick C.;
    • Revesz, Tamas;
    • Rossor, Martin N.
    Publication type:
    Article
    41

    A comparative clinical, pathological, biochemical and genetic study of fused in sarcoma proteinopathies.

    Published in:
    Brain: A Journal of Neurology, 2011, v. 134, n. 9, p. 2548, doi. 10.1093/brain/awr160
    By:
    • Lashley, Tammaryn;
    • Rohrer, Jonathan D.;
    • Bandopadhyay, Rina;
    • Fry, Charles;
    • Ahmed, Zeshan;
    • Isaacs, Adrian M.;
    • Brelstaff, Jack H.;
    • Borroni, Barbara;
    • Warren, Jason D.;
    • Troakes, Claire;
    • King, Andrew;
    • Al-Saraj, Safa;
    • Newcombe, Jia;
    • Quinn, Niall;
    • Ostergaard, Karen;
    • Schrøder, Henrik Daa;
    • Bojsen-Møller, Marie;
    • Braendgaard, Hans;
    • Fox, Nick C.;
    • Rossor, Martin N.
    Publication type:
    Article
    42
    43

    A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series.

    Published in:
    Brain: A Journal of Neurology, 2008, v. 131, n. 3, p. 706, doi. 10.1093/brain/awm320
    By:
    • Jonathan Beck;
    • Jonathan D. Rohrer;
    • Tracy Campbell;
    • Adrian Isaacs;
    • Karen E. Morrison;
    • Emily F. Goodall;
    • Elizabeth K. Warrington;
    • John Stevens;
    • Tamas Revesz;
    • Janice Holton;
    • Safa Al-Sarraj;
    • Andrew King;
    • Rachael Scahill;
    • Jason D. Warren;
    • Nick C. Fox;
    • Martin N. Rossor;
    • John Collinge;
    • Simon Mead
    Publication type:
    Article
    44
    45

    Association of missense and 5'-splice-site mutations in tau with the inherited dementia FTDP-17.

    Published in:
    Nature, 1998, v. 393, n. 6686, p. 702, doi. 10.1038/31508
    By:
    • Hutton, Mike;
    • Lendon, Corinne L.;
    • Rizzu, Patrizia;
    • Baker, Matt;
    • Froelich, Susanne;
    • Houlden, Henry;
    • Pickering-Brown, Stuart;
    • Chakraverty, Sumi;
    • Isaacs, Adrian;
    • Grover, Andrew;
    • Hackett, Jennifer;
    • Adamson, Jennifer;
    • Lincoln, Sarah;
    • Dickson, Dennis;
    • Davies, Peter;
    • Petersen, Ronald C.;
    • Stevens, Martijn;
    • de Graaff, Esther;
    • Wauters, Erwin;
    • van Baren, Jeltje
    Publication type:
    Article
    46
    47

    Evolution of the Aging Brain Transcriptome and Synaptic Regulation.

    Published in:
    PLoS ONE, 2008, v. 3, n. 10, p. 1, doi. 10.1371/journal.pone.0003329
    By:
    • Loerch, Patrick M.;
    • Tao Lu;
    • Dakin, Kelly A.;
    • Vann, James M.;
    • Isaacs, Adrian;
    • Geula, Chengiz;
    • Jianbin Wang;
    • Ying Pan;
    • Gabuzda, Dana H.;
    • Cheng Li;
    • Prolla, Tomas A.;
    • Yankner, Bruce A.
    Publication type:
    Article
    48

    Dysregulation in Subcellular Localization of Myelin Basic Protein mRNA Does Not Result in Altered Myelination in Amyotrophic Lateral Sclerosis.

    Published in:
    Frontiers in Neuroscience, 2021, v. 15, p. 1, doi. 10.3389/fnins.2021.705306
    By:
    • Barton, Samantha K.;
    • Gregory, Jenna M.;
    • Selvaraj, Bhuvaneish T.;
    • McDade, Karina;
    • Henstridge, Christopher M.;
    • Spires-Jones, Tara L.;
    • James, Owen G.;
    • Mehta, Arpan R.;
    • Story, David;
    • Burr, Karen;
    • Magnani, Dario;
    • Isaacs, Adrian M.;
    • Smith, Colin;
    • Chandran, Siddharthan
    Publication type:
    Article
    49

    Opinion: more mouse models and more translation needed for ALS.

    Published in:
    Molecular Neurodegeneration, 2023, v. 18, n. 1, p. 1, doi. 10.1186/s13024-023-00619-2
    By:
    • Fisher, Elizabeth M.C.;
    • Greensmith, Linda;
    • Malaspina, Andrea;
    • Fratta, Pietro;
    • Hanna, Michael G.;
    • Schiavo, Giampietro;
    • Isaacs, Adrian M.;
    • Orrell, Richard W.;
    • Cunningham, Thomas J.;
    • Arozena, Abraham Acevedo
    Publication type:
    Article
    50