C9orf72 amyotrophic lateral sclerosis and frontotemporal dementia: gain or loss of function?

por Mizielinska, Sarah, Isaacs, Adrian M.
Publicado 2014

The snowball effect of RNA binding protein dysfunction in amyotrophic lateral sclerosis

por Fratta, Pietro, Isaacs, Adrian M
Publicado 2018

Specific biomarkers for C9orf72 FTD/ALS could expedite the journey towards effective therapies

por Balendra, Rubika, Moens, Thomas G, Isaacs, Adrian M
Publicado 2017

Soluble and insoluble dipeptide repeat protein measurements in C9orf72-frontotemporal dementia brains show regional differential solubility and correlation of poly-GR with clinical...

por Quaegebeur, Annelies, Glaria, Idoia, Lashley, Tammaryn, Isaacs, Adrian M.
Publicado 2020

TDP-43 is a culprit in human neurodegeneration, and not just an innocent bystander

por Banks, Gareth T., Kuta, Anna, Isaacs, Adrian M., Fisher, Elizabeth M. C.
Publicado 2008

Quantitative analysis of cryptic splicing associated with TDP-43 depletion

por Humphrey, Jack, Emmett, Warren, Fratta, Pietro, Isaacs, Adrian M., Plagnol, Vincent
Publicado 2017

Glycine-alanine dipeptide repeats spread rapidly in a repeat length- and age-dependent manner in the fly brain

por Morón-Oset, Javier, Supèr, Tessa, Esser, Jacqueline, Isaacs, Adrian M., Grönke, Sebastian, Partridge, Linda
Publicado 2019

Heterogeneous nuclear ribonucleoproteins R and Q accumulate in pathological inclusions in FTLD-FUS

por Gittings, Lauren M., Foti, Sandrine C., Benson, Bridget C., Gami-Patel, Priya, Isaacs, Adrian M., Lashley, Tammaryn
Publicado 2019

C9orf72 hexanucleotide repeat associated with amyotrophic lateral sclerosis and frontotemporal dementia forms RNA G-quadruplexes

por Fratta, Pietro, Mizielinska, Sarah, Nicoll, Andrew J., Zloh, Mire, Fisher, Elizabeth M. C., Parkinson, Gary, Isaacs, Adrian M.
Publicado 2012

C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci

por Mizielinska, Sarah, Lashley, Tammaryn, Norona, Frances E., Clayton, Emma L., Ridler, Charlotte E., Fratta, Pietro, Isaacs, Adrian M.
Publicado 2013

Toxicity of C9orf72-associated dipeptide repeat peptides is modified by commonly used protein tags

por Morón-Oset, Javier, Fischer, Lilly KS, Carcolé, Mireia, Giblin, Ashling, Zhang, Pingze, Isaacs, Adrian M, Grönke, Sebastian, Partridge, Linda
Publicado 2023

Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration

por Mizielinska, Sarah, Ridler, Charlotte E., Balendra, Rubika, Thoeng, Annora, Woodling, Nathan S., Grässer, Friedrich A., Plagnol, Vincent, Lashley, Tammaryn, Partridge, Linda, Isaacs, Adrian M.
Publicado 2017

The integration site of the APP transgene in the J20 mouse model of Alzheimer’s disease

por Tosh, Justin L., Rickman, Matthew, Rhymes, Ellie, Norona, Frances E., Clayton, Emma, Mucke, Lennart, Isaacs, Adrian M., Fisher, Elizabeth M.C., Wiseman, Frances K.
Publicado 2018

Repeat length of C9orf72-associated glycine–alanine polypeptides affects their toxicity

por Morón-Oset, Javier, Fischer, Lilly Katharina Sophie, Jauré, Nathalie, Zhang, Pingze, Jahn, Annika Julia, Supèr, Tessa, Pahl, André, Isaacs, Adrian M., Grönke, Sebastian, Partridge, Linda
Publicado 2023

Frontotemporal dementia causative CHMP2B impairs neuronal endolysosomal traffic-rescue by TMEM106B knockdown

por Clayton, Emma L, Milioto, Carmelo, Muralidharan, Bhavana, Norona, Frances E, Edgar, James R, Soriano, Armand, Jafar-nejad, Paymaan, Rigo, Frank, Collinge, John, Isaacs, Adrian M
Publicado 2018

Opinion: more mouse models and more translation needed for ALS

por 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
Publicado 2023

Sense and antisense RNA are not toxic in Drosophila models of C9orf72-associated ALS/FTD

por Moens, Thomas G., Mizielinska, Sarah, Niccoli, Teresa, Mitchell, Jamie S., Thoeng, Annora, Ridler, Charlotte E., Grönke, Sebastian, Esser, Jacqueline, Heslegrave, Amanda, Zetterberg, Henrik, Partridge, Linda, Isaacs, Adrian M.
Publicado 2018

Symmetric dimethylation of poly-GR correlates with disease duration in C9orf72 FTLD and ALS and reduces poly-GR phase separation and toxicity

por Gittings, Lauren M., Boeynaems, Steven, Lightwood, Daniel, Clargo, Alison, Topia, Sarfaraj, Nakayama, Lisa, Troakes, Claire, Mann, David M. A., Gitler, Aaron D., Lashley, Tammaryn, Isaacs, Adrian M.
Publicado 2019

Sizing, stabilising, and cloning repeat-expansions for gene targeting constructs

por Nair, Remya R., Tibbit, Charlotte, Thompson, David, McLeod, Ross, Nakhuda, Asif, Simon, Michelle M., Baloh, Robert H., Fisher, Elizabeth M.C., Isaacs, Adrian M., Cunningham, Thomas J.
Publicado 2021

Conformation Determines the Seeding Potencies of Native and Recombinant Tau Aggregates

por Falcon, Benjamin, Cavallini, Annalisa, Angers, Rachel, Glover, Sarah, Murray, Tracey K., Barnham, Luanda, Jackson, Samuel, O'Neill, Michael J., Isaacs, Adrian M., Hutton, Michael L., Szekeres, Philip G., Goedert, Michel, Bose, Suchira
Publicado 2015