Publications by authors named "Adrian Isaacs"

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U7 small nuclear RNA splice-switching therapeutics for STMN2 and UNC13A in Amyotrophic Lateral Sclerosis.
Puja R Mehta, Tomas Solomon, Sarah Pickles, Peter Harley, Michela Barioglio, Adrian M Isaacs

bioRxiv· November 2025

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The Neurolipid Atlas: a lipidomics resource for neurodegenerative diseases.
Femke M Feringa, Sascha J Koppes-den Hertog, Lian Y Wang, Rico J E Derks, Iris Kruijff, Adrian M Isaacs

Nat Metab· October 2025

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An alternative cytoplasmic SFPQ isoform with reduced phase separation potential is up-regulated in ALS.
Jacob Neeves, Marija Petrić Howe, Oliver J Ziff, Beth Callaghan, Daniel Jutzi, Adrian M Isaacs

Sci Adv· August 2025

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Multi-modal comparative phenotyping of knock-in mouse models of frontotemporal dementia/amyotrophic lateral sclerosis.
Sevda Boyanova, Gareth Banks, Tatiana V Lipina, Rasneer Sonia Bains, Hamish Forrest, Adrian M Isaacs

Dis Model Mech· August 2025

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A multimodal screening platform for endogenous dipeptide repeat proteins in C9orf72 patient iPSC neurons.
Benedikt V Hölbling, Yashica Gupta, Paolo M Marchi, Magda L Atilano, Michael Flower, Adrian M Isaacs

Cell Rep· May 2025

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Neuronal polyunsaturated fatty acids are protective in ALS/FTD.
Ashling Giblin, Alexander J Cammack, Niek Blomberg, Sharifah Anoar, Alla Mikheenko, Adrian M Isaacs

Nat Neurosci· April 2025

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Author Correction: Neuronal polyunsaturated fatty acids are protective in ALS/FTD.
Ashling Giblin, Alexander J Cammack, Niek Blomberg, Sharifah Anoar, Alla Mikheenko, Adrian M Isaacs

Nat Neurosci· April 2025

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Differential neuronal vulnerability to C9orf72 repeat expansion driven by Xbp1-induced endoplasmic reticulum-associated degradation.
Dunxin Shen, Alec Vincent, Evan Udine, Yazead Buhidma, Sharifah Anoar, Adrian M Isaacs

Cell Rep· April 2025

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Amyotrophic lateral sclerosis caused by hexanucleotide repeat expansions in C9orf72: from genetics to therapeutics.
Sarah Mizielinska, Guillaume M Hautbergue, Tania F Gendron, Marka van Blitterswijk, Orla Hardiman, Adrian M Isaacs

Lancet Neurol· March 2025

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Generation of repeat knock-in iPSC lines for modelling ALS and FTD.
Rachel Coneys, Alexander J Cammack, Remya R Nair, David Thompson, Jonas Mechtersheimer, Adrian M Isaacs

bioRxiv· February 2025

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Dual-targeting CRISPR-CasRx reduces C9orf72 ALS/FTD sense and antisense repeat RNAs in vitro and in vivo.
Liam Kempthorne, Deniz Vaizoglu, Alexander J Cammack, Mireia Carcolé, Martha J Roberts, Adrian M Isaacs

Nat Commun· January 2025

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Poly-GR Impairs PRMT1-Mediated Arginine Methylation of Disease-Linked RNA-Binding Proteins by Acting as a Substrate Sink.
Saskia Hutten, Jia-Xuan Chen, Adrian M Isaacs, Dorothee Dormann

Biochemistry· September 2024

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Molecular pathology, developmental changes and synaptic dysfunction in (pre-) symptomatic human C9ORF72-ALS/FTD cerebral organoids.
Astrid T van der Geest, Channa E Jakobs, Tijana Ljubikj, Christiaan F M Huffels, Marta Cañizares Luna, Adrian M Isaacs

Acta Neuropathol Commun· September 2024

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Failure of C9orf72 sense repeat-targeting antisense oligonucleotides: lessons learned and the path forward.
Alexander J Cammack, Rubika Balendra, Adrian M Isaacs

Brain· August 2024

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Cellular and axonal transport phenotypes due to the C9ORF72 HRE in iPSC motor and sensory neurons.
Jakub Scaber, Iona Thomas-Wright, Alex J Clark, Yinyan Xu, Björn F Vahsen, Adrian M Isaacs

Stem Cell Reports· July 2024

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The Neurolipid Atlas: a lipidomics resource for neurodegenerative diseases uncovers cholesterol as a regulator of astrocyte reactivity impaired by ApoE4.
Femke M Feringa, Sascha J Koppes-den Hertog, Lian Wang, Rico J E Derks, Iris Kruijff, Adrian M Isaacs

bioRxiv· July 2024

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PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in C9orf72 ALS/FTD neurons.
Carmelo Milioto, Mireia Carcolé, Ashling Giblin, Rachel Coneys, Olivia Attrebi, Adrian M Isaacs

Nat Neurosci· April 2024

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GC targeting antisense oligonucleotides potently mitigate TDP-43 dysfunction in human C9orf72 ALS/FTD induced pluripotent stem cell derived neurons.
Jeffrey D Rothstein, Victoria Baskerville, Sampath Rapuri, Emma Mehlhop, Paymaan Jafar-Nejad, Adrian Isaacs

Acta Neuropathol· November 2023

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Toxicity of -associated dipeptide repeat peptides is modified by commonly used protein tags.
Javier Morón-Oset, Lilly Ks Fischer, Mireia Carcolé, Ashling Giblin, Pingze Zhang, Adrian M Isaacs

Life Sci Alliance· September 2023

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Transcriptome-wide RNA binding analysis of C9orf72 poly(PR) dipeptides.
Rubika Balendra, Igor Ruiz de Los Mozos, Hana M Odeh, Idoia Glaria, Carmelo Milioto, Adrian M Isaacs

Life Sci Alliance· September 2023

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A monocarboxylate transporter rescues frontotemporal dementia and Alzheimer's disease models.
Dongwei Xu, Alec Vincent, Andrés González-Gutiérrez, Benjamin Aleyakpo, Sharifah Anoar, Adrian M Isaacs

PLoS Genet· September 2023

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C9orf72-ALS human iPSC microglia are pro-inflammatory and toxic to co-cultured motor neurons via MMP9.
Björn F Vahsen, Sumedha Nalluru, Georgia R Morgan, Lucy Farrimond, Emily Carroll, Adrian M Isaacs

Nat Commun· September 2023

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Repeat length of C9orf72-associated glycine-alanine polypeptides affects their toxicity.
Javier Morón-Oset, Lilly Katharina Sophie Fischer, Nathalie Jauré, Pingze Zhang, Annika Julia Jahn, Adrian M Isaacs

Acta Neuropathol Commun· August 2023

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Opinion: more mouse models and more translation needed for ALS.
Elizabeth M C Fisher, Linda Greensmith, Andrea Malaspina, Pietro Fratta, Michael G Hanna, Adrian M Isaacs

Mol Neurodegener· May 2023

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Development of a sensitive trial-ready poly(GP) CSF biomarker assay for -associated frontotemporal dementia and amyotrophic lateral sclerosis.
Katherine M Wilson, Eszter Katona, Idoia Glaria, Mireia Carcolé, Imogen J Swift, Adrian M Isaacs

J Neurol Neurosurg Psychiatry· July 2022

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