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C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins
TLDR
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
TLDR
Lowered activity of the nutrient-sensing insulin/insulin-like growth factor/Target of Rapamycin signalling network can extend healthy lifespan in yeast, multicellular invertebrates, mice and, possibly, humans.
Capu and Spire Assemble a Cytoplasmic Actin Mesh that Maintains Microtubule Organization in the Drosophila Oocyte
TLDR
The Capu pathway controls alternative states of the oocyte cytoplasm: when active, it assembles an actin mesh that suppresses kinesin motility to maintain a polarized microtubule cytoskeleton, and regulates microtubules indirectly, by inhibiting kinesIn-dependent cy toplasmic flows.
Tea3p Is a Cell End Marker Activating Polarized Growth in Schizosaccharomyces pombe
TLDR
It is proposed that Tea3p is a novel cell end marker required specifically to activate polarized cell growth at the second end during NETO, which is required for efficient NETO and for the proper placement of the septum.
The p150-Glued Ssm4p regulates microtubular dynamics and nuclear movement in fission yeast
TLDR
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.
G‐quadruplex‐binding small molecules ameliorate C9orf72 FTD/ALS pathology in vitro and in vivo
TLDR
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.
RPS25 is required for efficient RAN translation of C9orf72 and other neurodegenerative disease-associated nucleotide repeats
TLDR
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.
C9orf72 arginine-rich dipeptide proteins interact with ribosomal proteins in vivo to induce a toxic translational arrest that is rescued by eIF1A
TLDR
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.
Cell-Nonautonomous Effects of dFOXO/DAF-16 in Aging
TLDR
In Drosophila, activation of dfoxo/daf-16 in the gut/fat body or in neuroendocrine cells acts on other organs to promote healthy aging by signaling to other, as-yet-unidentified factors.
Sense and antisense RNA are not toxic in Drosophila models of C9orf72-associated ALS/FTD
TLDR
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.
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