A5082077990- Amyotrophic Lateral Sclerosis Research
- Neurogenetic and Muscular Disorders Research
- Alzheimer's disease research and treatments
- Pluripotent Stem Cells Research
- Ion channel regulation and function
- CRISPR and Genetic Engineering
- Neuroscience and Neural Engineering
- Muscle Physiology and Disorders
- Pharmacological Receptor Mechanisms and Effects
- Neuroinflammation and Neurodegeneration Mechanisms
- Mitochondrial Function and Pathology
- biodegradable polymer synthesis and properties
- Hedgehog Signaling Pathway Studies
- Prion Diseases and Protein Misfolding
- Nerve injury and regeneration
- Neuroscience and Neuropharmacology Research
- Organic Light-Emitting Diodes Research
- Extracellular vesicles in disease
- Nicotinic Acetylcholine Receptors Study
- Axon Guidance and Neuronal Signaling
- Neurogenesis and neuroplasticity mechanisms
- Biomedical Ethics and Regulation
- Autophagy in Disease and Therapy
- Neurological diseases and metabolism
- Semiconductor materials and devices
KU Leuven
2019-2025
VIB-KU Leuven Center for Brain & Disease Research
2019-2025
Allen Institute for Brain Science
2021-2024
University of Copenhagen
2021-2024
Abstract Background Astrocytes play a crucial, yet not fully elucidated role in the selective motor neuron pathology amyotrophic lateral sclerosis (ALS). Among other responsibilities, astrocytes provide important neuronal homeostatic support, however this function is highly compromised ALS. The establishment of human coculture systems can be used to further study underlying mechanisms dysfunctional intercellular interplay, and has potential platform for revealing novel therapeutic entry...
Neuromuscular junctions (NMJs) ensure communication between motor neurons (MNs) and muscle; however, in MN disorders, such as amyotrophic lateral sclerosis (ALS), NMJs degenerate resulting muscle atrophy. The aim of this study was to establish a versatile reproducible vitro model human unit investigate the effects ALS-causing mutations. Therefore, we generated co-culture induced pluripotent stem cell (iPSC)-derived MNs primary mesoangioblast-derived myotubes microfluidic devices. A...
Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which currently lacks effective treatments. Mutations in the RNA-binding protein FUS are common cause of familial ALS, accounting for around 4% cases. Understanding mechanisms by mutant becomes toxic to neurons can provide insight into pathogenesis both and sporadic ALS. We have previously observed that overexpression wild-type or ALS-mutant Drosophila motor toxic, allowed us screen novel genetic modifiers...
Frontotemporal dementia type 3 (FTD3), caused by a point mutation in the charged multivesicular body protein 2B (CHMP2B), affects mitochondrial ultrastructure and endolysosomal pathway neurons. To dissect astrocyte-specific impact of mutant CHMP2B expression, we generated astrocytes from human induced pluripotent stem cells (hiPSCs) confirmed our findings mice. Our data provide mechanistic insights into how defective autophagy causes perturbed dynamics with impaired glycolysis, increased...
Upregulation of L-type calcium channels (LTCCs) is implicated in a range cardiovascular and neurological disorders. Therefore, the development toolboxes that unlocks fast imaging protocols live cells are coveted. Herein, we report library first-in-class novel far-red small-molecule-based fluorescent ligands (FluoDiPines), able to target LTCCs. All were evaluated whole-cell patch-clamp live-cell Ca2+ whereby Fluodipine 6 was found best candidate for fluorescence imaging. Low concentration...
Neuromuscular junctions (NMJs) are specialized synapses, crucial for the communication between spinal motor neurons (MNs) and skeletal muscle. NMJs become vulnerable in degenerative diseases, such as muscle atrophy, where crosstalk different cell populations fails, regenerative ability of entire tissue is hampered. How sends retrograde signals to MNs through represents an intriguing field research, role oxidative stress its sources remain poorly understood. Recent works demonstrate myofiber...
Key features• This protocol requires preexisting experience in hiPSC culturing for a successful outcome.• The relies on small molecule differentiation scheme and an easy-to-follow methodology, which can be paused at several time points.• generates >50 × 10 6 astrocytes per differentiation, cryopreserved every passage, ensuring large-scale experimental output.
Abstract Progressive loss of motor neurons is the hallmark neurodegenerative disease amyotrophic lateral sclerosis (ALS), but underlying mechanisms remain incompletely understood. In this study, we investigate effects C21ORF2 mutations, a gene recently linked to ALS, and find that primary cilia are dysfunctional. Human patient-derived mutant have reduced ciliary frequency length. We report located at basal body cilium, mutations associated with ALS alter localization. Furthermore, show...
Neuromuscular junctions (NMJs) are specialized synapses between the axon of lower motor neuron and muscle facilitating engagement contraction. In disorders, such as amyotrophic lateral sclerosis (ALS) spinal muscular atrophy (SMA), NMJs degenerate, resulting in progressive paralysis. The underlying mechanism NMJ degeneration is unknown, largely due to lack translatable research models. This study aimed create a versatile reproducible vitro model human unit with functional NMJs. Therefore,...
The veterinary and animal science professions are rapidly developing their inherent historical connection to agriculture is challenged by more biomedical medical directions of research. While some consider this development as a risk losing identity, it may also be seen an opportunity for further sophisticated competences that ultimately feed back in synergistic way. present review describes how agriculture-related studies on bovine vitro embryo production through putative porcine embryonic...
Neuromuscular junctions (NMJs) are specialized synapses between the axon of lower motor neuron and muscle facilitating engagement contraction. In disorders, such as amyotrophic lateral sclerosis (ALS) spinal muscular atrophy (SMA), NMJs degenerate, resulting in progressive paralysis. The underlying mechanism NMJ degeneration is unknown, largely due to lack translatable research models. This study aimed create a versatile reproducible vitro model human unit with functional NMJs. Therefore,...
Upregulation of L-type calcium channels (LTCCs) is implicated in a range cardiovascular and neurological disorders. Therefore, the development toolboxes that unlocks fast imaging protocols live cells are coveted. Herein, we report library first-in-class novel far-red small-molecule-based fluorescent ligands (FluoDiPines), able to target LTCCs. All were evaluated whole-cell patch-clamp live-cell Ca2+ whereby Fluodipine 6 was found best candidate for fluorescence imaging. Low concentration...
Abstract Neuromuscular junctions (NMJs) ensure proper communication between motor neurons and muscle through the release of neurotransmitters. In neuron disorders, such as amyotrophic lateral sclerosis (ALS), NMJs degenerate resulting in atrophy, paralysis respiratory failure. The aim this study was to establish a versatile reproducible vitro model human unit effect ALS-causing mutations. Therefore, we generated co-culture induced pluripotent stem cell-derived primary mesoangioblast-derived...
Upregulation of L-type calcium channels (LTCCs) is implicated in a range cardiovascular and neurological disorders. Therefore, the development toolboxes that unlock fast imaging protocols live cells coveted. Herein, we report library first-in-class far-red small-molecule-based fluorescent ligands (FluoDiPines), able to target LTCCs. All were evaluated whole-cell patch-clamp live-cell Ca
Abstract ALS is a fatal neurodegenerative disease which currently lacks effective treatments. Mutations in the RNA-binding protein FUS are common cause of familial ALS, accounting for around 4% fALS cases. Studying mechanisms by mutant toxic to neurons may provide insight into pathogenesis both and sporadic forms ALS. Here we identify Protein Phosphatase 2A (PP2A) Glycogen Synthase Kinase 3 (GSK3) as novel modifiers FUS-ALS vivo , looking from fly human. PP2A-C GSK3β inhibition rescued...
Abstract Background : Frontotemporal dementia type 3 (FTD3) caused by a point mutation in the charged multivesicular body protein 2B (CHMP2B), affects mitochondrial ultrastructure and function as well endosomal-lysosomal fusion neurons. However, there is critical knowledge gap understanding how mutations CHMP2B affect astrocytes. Hence, we investigated disease mechanisms astrocytes derived from hiPSC with their impact on Methods To dissect astrocyte-specific of mutant expression, generated...