C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins
- S. Mizielinska, Sebastian Grönke, A. Isaacs
- BiologyScience
- 5 September 2014
In vitro and in vivo models to dissect repeat RNA and dipeptide repeat protein toxicity are developed, consistent with a dual toxicity mechanism, whereby both arginine-rich proteins and repeat RNA contribute to C9orf72-mediated neurodegeneration.
Ageing as a Risk Factor for Disease
- Teresa Niccoli, L. Partridge
- BiologyCurrent Biology
- 11 September 2012
Capu and Spire Assemble a Cytoplasmic Actin Mesh that Maintains Microtubule Organization in the Drosophila Oocyte
- K. Dahlgaard, A. Raposo, Teresa Niccoli, D. St Johnston
- BiologyDevelopmental Cell
- 9 October 2007
Tea3p Is a Cell End Marker Activating Polarized Growth in Schizosaccharomyces pombe
- M. Arellano, Teresa Niccoli, P. Nurse
- BiologyCurrent Biology
- 30 April 2002
C9orf72 arginine-rich dipeptide proteins interact with ribosomal proteins in vivo to induce a toxic translational arrest that is rescued by eIF1A
- T. Moens, Teresa Niccoli, A. Isaacs
- BiologyActa Neuropathologica
- 2 January 2019
expression of the translation initiation factor eIF1A uniquely rescued DPR-induced toxicity in vivo, indicating that restoring translation is a potential therapeutic strategy and directly implicate translational repression in C9orf72 repeat-induced neurodegeneration.
G‐quadruplex‐binding small molecules ameliorate C9orf72 FTD/ALS pathology in vitro and in vivo
- Roberto Simone, R. Balendra, A. Isaacs
- BiologyEMBO Molecular Medicine
- 7 November 2017
Data provide proof of principle that targeting GGGGCC repeat G‐quadruplexes has therapeutic potential and can ameliorate the two key pathologies associated with C9orf72 FTD/ALS.
Sense and antisense RNA are not toxic in Drosophila models of C9orf72-associated ALS/FTD
- T. Moens, S. Mizielinska, A. Isaacs
- BiologyActa Neuropathologica
- 29 January 2018
It is found that neither cytoplasmic nor nuclear sense or antisense RNA are toxic when expressed in adult Drosophila neurons, suggesting they have a limited role in disease pathogenesis.
Ageing as a risk factor for ALS/FTD
- Teresa Niccoli, L. Partridge, A. Isaacs
- BiologyHuman Molecular Genetics
- 1 October 2017
Some of the shared mechanisms between the ageing process itself and emerging pathogenic mechanisms in ALS/FTD are highlighted.
RPS25 is required for efficient RAN translation of C9orf72 and other neurodegenerative disease-associated nucleotide repeats
- Shizuka Yamada, T. Gendron, A. Gitler
- BiologyNature Neuroscience
- 21 June 2019
A genetic screen for regulators of RAN translation is performed and small ribosomal protein subunit 25 (RPS25) is identified, presenting a potential therapeutic target for C9orf72-related amyotrophic lateral sclerosis and frontotemporal dementia and other neurodegenerative diseases caused by nucleotide repeat expansions.
The p150-Glued Ssm4p regulates microtubular dynamics and nuclear movement in fission yeast
- Teresa Niccoli, A. Yamashita, P. Nurse, Masayuki Yamamoto
- BiologyJournal of Cell Science
- 1 November 2004
It is shown that Ssm4p, a p150-Glued protein, is induced specifically in response to pheromone and is required for this nuclear movement after cellular and nuclear fusion in the zygote and together with the CLIP-170 homologue Tip1p regulates dynein heavy chain localisation.
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