Ludo Van Den Bosch

The paper introduces MeFluHyA, a fluorescent probe for identifying HDAC6-selective inhibitors through cell imaging and competitive binding assays.

CREsted is a software package for modeling and designing cell type-specific enhancers using deep learning, validated across tissues and species.

This study shows that fructose-2,6-bisphosphate (F2,6BP) restores genome repair and motor function in TDP-43 pathology-driven motor neuron diseases.

This paper introduces human myelin spheres (MyS) as a model for studying myelination and demyelination, showing their ability to form myelinated neurons and mimic in vivo oligodendrocyte development.

The paper creates a single-nucleus transcriptomic atlas of oligodendrocytes in ALS, identifying disease-associated activation states and potential biomarkers.

This study demonstrates that CRISPR-CasRx reduces C9orf72 repeat RNAs and DPRs in vitro and in vivo, suggesting its therapeutic potential for ALS/FTD.

This study investigates the role of oligodendroglia in Wolfram syndrome using mouse models and iPSC-derived cells, finding preserved oligodendroglia function and suggesting alternative targets for treatment.

This study identifies PP2A and GSK3 as modifiers of FUS-ALS by modulating mitochondrial transport in Drosophila and human iPSC-derived motor neurons.

This study shows that polystyrene sulfonate (PSS) reduces toxicity of ALS-linked dipeptide repeat proteins in cellular and iPSC-derived motor neuron models.

This study identifies that antisense C4G2 repeat expanded RNAs, but not sense G4C2, activate the PKR/eIF2-dependent integrated stress response in C9orf72 FTD/ALS, leading to neuronal toxicity.

This study identifies defective lipid metabolism in Schwann cells as a cause of CMT1A, showing altered lipid composition and membrane dynamics in mouse models and human iPSC-derived cells.

This study shows that C9orf72 hexanucleotide repeat expansions impair microglial function and disrupt glial communication in ALS, contributing to disease risk.

This study identifies C21orf2 mutations in ALS as causing cilia dysfunction and impaired sonic hedgehog signaling in motor neurons.

This study uses a microfluidic model of human motor units to show that FUS mutations impair neurite outgrowth and NMJ formation, which can be mitigated by HDAC6 inhibition.

This study shows that C9orf72 repeat expansions produce toxic arginine-rich DPRs that disrupt axonal transport by interacting with microtubules and motor proteins, contributing to ALS/FTD.

The paper introduces a method to study liquid-liquid phase separation under near-native conditions by using extreme pH to control protein solubility and induce phase separation.

This study uses APEX proximity-proteomics to analyze the spatio-temporal proteomic changes during stress granule disassembly, identifying novel proteins and revealing SUMOylation's role in ALS pathogenesis.