Ludo Van Den Bosch Biography Publications JoVE Articles Ludo Van Den Bosch has not added a biography. If you are Ludo Van Den Bosch and would like to personalize this page please email our Author Liaison for assistance. Publications FUS-ALS HiPSC-derived Astrocytes Impair Human Motor Units Through Both Gain-of-toxicity and Loss-of-support Mechanisms Molecular Neurodegeneration. Jan, 2023 | Pubmed ID: 36653804 Downregulation of PMP22 Ameliorates Myelin Defects in IPSC-derived Human Organoid Cultures of CMT1A Brain : a Journal of Neurology. Dec, 2022 | Pubmed ID: 36511878 CRISPR/Cas9 Screen in Human IPSC-derived Cortical Neurons Identifies NEK6 As a Novel Disease Modifier of C9orf72 Poly(PR) Toxicity Alzheimer's & Dementia : the Journal of the Alzheimer's Association. Aug, 2022 | Pubmed ID: 35993441 HNRNPK Alleviates RNA Toxicity by Counteracting DNA Damage in C9orf72 ALS Acta Neuropathologica. Sep, 2022 | Pubmed ID: 35895140 Increased Pyroptosis Activation in White Matter Microglia is Associated with Neuronal Loss in ALS Motor Cortex Acta Neuropathologica. Sep, 2022 | Pubmed ID: 35867112 ALS-associated KIF5A Mutations Abolish Autoinhibition Resulting in a Toxic Gain of Function Cell Reports. Apr, 2022 | Pubmed ID: 35385738 Frontotemporal Lobar Degeneration Case with an N-Terminal Mutation Exhibits Reduced TUBA4A Levels in the Brain and TDP-43 Pathology Biomolecules. Mar, 2022 | Pubmed ID: 35327632 HDAC3 Inhibition Stimulates Myelination in a CMT1A Mouse Model Molecular Neurobiology. Jun, 2022 | Pubmed ID: 35320455 The Role of Nucleocytoplasmic Transport Defects in Amyotrophic Lateral Sclerosis International Journal of Molecular Sciences. Nov, 2021 | Pubmed ID: 34830069 Histone Deacetylase Inhibition Regulates Lipid Homeostasis in a Mouse Model of Amyotrophic Lateral Sclerosis International Journal of Molecular Sciences. Oct, 2021 | Pubmed ID: 34681883 Potential Therapeutic Role of HDAC Inhibitors in FUS-ALS Frontiers in Molecular Neuroscience. 2021 | Pubmed ID: 34434087 Induced Pluripotent Stem Cell-derived Motor Neurons of CMT Type 2 Patients Reveal Progressive Mitochondrial Dysfunction Brain : a Journal of Neurology. Jun, 2021 | Pubmed ID: 34128983 Exploring the Alternative: Fish, Flies and Worms As Preclinical Models for ALS Neuroscience Letters. 08, 2021 | Pubmed ID: 34118308 Reply to 'TDP43 Aggregates: the 'Schrödinger's Cat' in Amyotrophic Lateral Sclerosis' Nature Reviews. Neuroscience. Aug, 2021 | Pubmed ID: 34103711 Liquid-Liquid Phase Separation Enhances TDP-43 LCD Aggregation but Delays Seeded Aggregation Biomolecules. 04, 2021 | Pubmed ID: 33917983 Human Motor Units in Microfluidic Devices Are Impaired by FUS Mutations and Improved by HDAC6 Inhibition Stem Cell Reports. Apr, 2021 | Pubmed ID: 33891869 -derived Arginine-containing Dipeptide Repeats Associate with Axonal Transport Machinery and Impede Microtubule-based Motility Science Advances. Apr, 2021 | Pubmed ID: 33837088 HDAC6 Inhibition Restores TDP-43 Pathology and Axonal Transport Defects in Human Motor Neurons with TARDBP Mutations The EMBO Journal. Apr, 2021 | Pubmed ID: 33694180 AAV9-mediated Gene Delivery of MCT1 to Oligodendrocytes Does Not Provide a Therapeutic Benefit in a Mouse Model of ALS Molecular Therapy. Methods & Clinical Development. Mar, 2021 | Pubmed ID: 33614825 Opportunities for Histone Deacetylase Inhibition in Amyotrophic Lateral Sclerosis British Journal of Pharmacology. 03, 2021 | Pubmed ID: 32726472 Quantitative Nucleocytoplasmic Transport Assays in Cellular Models of Neurodegeneration Bio-protocol. Jun, 2020 | Pubmed ID: 33659329 Spatiotemporal Proteomic Analysis of Stress Granule Disassembly Using APEX Reveals Regulation by SUMOylation and Links to ALS Pathogenesis Molecular Cell. 12, 2020 | Pubmed ID: 33217318 Repeat Expansions Confer Risk for Amyotrophic Lateral Sclerosis and Contribute to TDP-43 Mislocalization Brain Communications. 2020 | Pubmed ID: 32954321 C9orf72 Loss-of-function: a Trivial, Stand-alone or Additive Mechanism in C9 ALS/FTD? Acta Neuropathologica. 11, 2020 | Pubmed ID: 32876811 The Multifaceted Role of Kinases in Amyotrophic Lateral Sclerosis: Genetic, Pathological and Therapeutic Implications Brain : a Journal of Neurology. 06, 2020 | Pubmed ID: 32206784 HDAC6 Inhibitors: Translating Genetic and Molecular Insights into a Therapy for Axonal CMT Brain Research. 04, 2020 | Pubmed ID: 32006555 RNA Toxicity in Non-coding Repeat Expansion Disorders The EMBO Journal. 01, 2020 | Pubmed ID: 31721251 Axonal Transport Defects and Neurodegeneration: Molecular Mechanisms and Therapeutic Implications Seminars in Cell & Developmental Biology. 03, 2020 | Pubmed ID: 31542222 EphA4 Loss Improves Social Memory Performance and Alters Dendritic Spine Morphology Without Changes in Amyloid Pathology in a Mouse Model of Alzheimer's Disease Alzheimer's Research & Therapy. 12, 2019 | Pubmed ID: 31831046 Existing and Emerging Metabolomic Tools for ALS Research Genes. 12, 2019 | Pubmed ID: 31817338 C9orf72-generated Poly-GR and Poly-PR Do Not Directly Interfere with Nucleocytoplasmic Transport Scientific Reports. 10, 2019 | Pubmed ID: 31673013 Reducing EphA4 Before Disease Onset Does Not Affect Survival in a Mouse Model of Amyotrophic Lateral Sclerosis Scientific Reports. 10, 2019 | Pubmed ID: 31575928 Reduction of Ephrin-A5 Aggravates Disease Progression in Amyotrophic Lateral Sclerosis Acta Neuropathologica Communications. 07, 2019 | Pubmed ID: 31300041 Restoration of Histone Acetylation Ameliorates Disease and Metabolic Abnormalities in a FUS Mouse Model Acta Neuropathologica Communications. 07, 2019 | Pubmed ID: 31277703 Altered Calcium Dynamics and Glutamate Receptor Properties in IPSC-derived Motor Neurons from ALS Patients with C9orf72, FUS, SOD1 or TDP43 Mutations Human Molecular Genetics. 09, 2019 | Pubmed ID: 31108504 FUS-induced Neurotoxicity in Drosophila is Prevented by Downregulating Nucleocytoplasmic Transport Proteins Human Molecular Genetics. 12, 2018 | Pubmed ID: 30379317 HDAC6 As a Potential Therapeutic Target for Peripheral Nerve Disorders Expert Opinion on Therapeutic Targets. 12, 2018 | Pubmed ID: 30360671 Progranulin Reduces Insoluble TDP-43 Levels, Slows Down Axonal Degeneration and Prolongs Survival in Mutant TDP-43 Mice Molecular Neurodegeneration. 10, 2018 | Pubmed ID: 30326935 Molecular Dissection of FUS Points at Synergistic Effect of Low-Complexity Domains in Toxicity Cell Reports. 07, 2018 | Pubmed ID: 30021151 Astrocyte-derived Jagged-1 Mitigates Deleterious Notch Signaling in Amyotrophic Lateral Sclerosis Neurobiology of Disease. 11, 2018 | Pubmed ID: 30010003 Conditional Deletion of Id2 or Notch1 in Oligodendrocyte Progenitor Cells Does Not Ameliorate Disease Outcome in SOD1 Mice Neurobiology of Aging. Aug, 2018 | Pubmed ID: 29689424 Protein Phase Separation: A New Phase in Cell Biology Trends in Cell Biology. Jun, 2018 | Pubmed ID: 29602697 Genome-wide Analyses Identify KIF5A As a Novel ALS Gene Neuron. Mar, 2018 | Pubmed ID: 29566793 Energy Metabolism in ALS: an Underappreciated Opportunity? Acta Neuropathologica. Apr, 2018 | Pubmed ID: 29549424 Phasing in on the Cell Cycle Cell Division. 2018 | Pubmed ID: 29416553 HDAC6 is a Therapeutic Target in Mutant GARS-induced Charcot-Marie-Tooth Disease Brain : a Journal of Neurology. Feb, 2018 | Pubmed ID: 29415205 Elongator Subunit 3 (ELP3) Modifies ALS Through TRNA Modification Human Molecular Genetics. Apr, 2018 | Pubmed ID: 29415125 A Zebrafish Model for C9orf72 ALS Reveals RNA Toxicity As a Pathogenic Mechanism Acta Neuropathologica. Mar, 2018 | Pubmed ID: 29302778 Inhibition of Histone Deacetylase 6 (HDAC6) Protects Against Vincristine-induced Peripheral Neuropathies and Inhibits Tumor Growth Neurobiology of Disease. Mar, 2018 | Pubmed ID: 29197621 Arginine-rich Peptides Can Actively Mediate Liquid-liquid Phase Separation Bio-protocol. Sep, 2017 | Pubmed ID: 34541184 HDAC6 Inhibition Reverses Axonal Transport Defects in Motor Neurons Derived from FUS-ALS Patients Nature Communications. 10, 2017 | Pubmed ID: 29021520 Identification and Characterization of Nanobodies Targeting the EphA4 Receptor The Journal of Biological Chemistry. 07, 2017 | Pubmed ID: 28526745 Modelling Amyotrophic Lateral Sclerosis: Progress and Possibilities Disease Models & Mechanisms. May, 2017 | Pubmed ID: 28468939 Progranulin Functions As a Cathepsin D Chaperone to Stimulate Axonal Outgrowth in Vivo Human Molecular Genetics. 08, 2017 | Pubmed ID: 28453791 Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics Molecular Cell. Mar, 2017 | Pubmed ID: 28306503 Defective Axonal Transport: A Common Pathological Mechanism in Inherited and Acquired Peripheral Neuropathies Neurobiology of Disease. Sep, 2017 | Pubmed ID: 28238949 Genetic Ablation of IP3 Receptor 2 Increases Cytokines and Decreases Survival of SOD1G93A Mice Human Molecular Genetics. 08, 2016 | Pubmed ID: 27378687 Inside Out: the Role of Nucleocytoplasmic Transport in ALS and FTLD Acta Neuropathologica. Aug, 2016 | Pubmed ID: 27271576 Drosophila Screen Connects Nuclear Transport Genes to DPR Pathology in C9ALS/FTD Scientific Reports. Feb, 2016 | Pubmed ID: 26869068 Guidelines for the Use and Interpretation of Assays for Monitoring Autophagy (3rd Edition) Autophagy. Jan, 2016 | Pubmed ID: 26799652 Modifiers of C9orf72 Dipeptide Repeat Toxicity Connect Nucleocytoplasmic Transport Defects to FTD/ALS Nature Neuroscience. Sep, 2015 | Pubmed ID: 26308983 The Role of Oligodendroglial Dysfunction in Amyotrophic Lateral Sclerosis Neurodegenerative Disease Management. 2014 | Pubmed ID: 25095817 Prevention of Intestinal Obstruction Reveals Progressive Neurodegeneration in Mutant TDP-43 (A315T) Mice Molecular Neurodegeneration. Jun, 2014 | Pubmed ID: 24938805 Role and Therapeutic Potential of Liquid‒liquid Phase Separation in Amyotrophic Lateral Sclerosis Journal of Molecular Cell Biology. | Pubmed ID: 32976566 Lowering EphA4 Does Not Ameliorate Disease in a Mouse Model for Severe Spinal Muscular Atrophy Frontiers in Neuroscience. | Pubmed ID: 31803009 Differentiation but Not ALS Mutations in FUS Rewires Motor Neuron Metabolism Nature Communications. | Pubmed ID: 31515480 Current Methods In ALS Research JoVE 65016 Editorial 미세 유체 장치에서 기능성 신경 근육 접합을 갖춘 인간 모터 유닛 생성 Katarina Stoklund Dittlau1,2, Emily N. Krasnow1,2, Laura Fumagalli1,2, Tijs Vandoorne1,2, Pieter Baatsen3,4, Axelle Kerstens3,4, Giorgia Giacomazzi5, Benjamin Pavie3,4, Elisabeth Rossaert1,2, Jimmy Beckers1,2, Maurilio Sampaolesi5, Philip Van Damme1,2,6, Ludo Van Den Bosch1,2 1Department of Neurosciences, Experimental Neurology, and Leuven Brain Institute, KU Leuven - University of Leuven, 2VIB Center for Brain & Disease Research, Laboratory of Neurobiology, , 3VIB Center for Brain & Disease Research, Research Group Molecular Neurobiology, , 4VIB Bio Imaging Core, KU Leuven - University of Leuven, 5Department of Development and Regeneration, Stem Cell and Developmental Biology, KU Leuven - University of Leuven, 6Department of Neurology, University Hospitals Leuven JoVE 62959 Neuroscience Vivo에서 모터 신경 퇴보의 마우스 모델에서 앞 발에서 복합 근육 활동 전위의 electrophysiological 측정 Eveliina Pollari1,2, Robert Prior1,2, Wim Robberecht1,2,3, Philip Van Damme1,2,3, Ludo Van Den Bosch1,2 1Department of Neurosciences, Experimental Neurology, KU Leuven - University of Leuven, 2Center for Brain & Disease Research, Laboratory of Neurobiology, VIB, 3Department of Neurology, University Hospitals Leuven JoVE 57741 Neuroscience
미세 유체 장치에서 기능성 신경 근육 접합을 갖춘 인간 모터 유닛 생성 Katarina Stoklund Dittlau1,2, Emily N. Krasnow1,2, Laura Fumagalli1,2, Tijs Vandoorne1,2, Pieter Baatsen3,4, Axelle Kerstens3,4, Giorgia Giacomazzi5, Benjamin Pavie3,4, Elisabeth Rossaert1,2, Jimmy Beckers1,2, Maurilio Sampaolesi5, Philip Van Damme1,2,6, Ludo Van Den Bosch1,2 1Department of Neurosciences, Experimental Neurology, and Leuven Brain Institute, KU Leuven - University of Leuven, 2VIB Center for Brain & Disease Research, Laboratory of Neurobiology, , 3VIB Center for Brain & Disease Research, Research Group Molecular Neurobiology, , 4VIB Bio Imaging Core, KU Leuven - University of Leuven, 5Department of Development and Regeneration, Stem Cell and Developmental Biology, KU Leuven - University of Leuven, 6Department of Neurology, University Hospitals Leuven JoVE 62959 Neuroscience
Vivo에서 모터 신경 퇴보의 마우스 모델에서 앞 발에서 복합 근육 활동 전위의 electrophysiological 측정 Eveliina Pollari1,2, Robert Prior1,2, Wim Robberecht1,2,3, Philip Van Damme1,2,3, Ludo Van Den Bosch1,2 1Department of Neurosciences, Experimental Neurology, KU Leuven - University of Leuven, 2Center for Brain & Disease Research, Laboratory of Neurobiology, VIB, 3Department of Neurology, University Hospitals Leuven JoVE 57741 Neuroscience
