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Showing results 1 to 20 of 26
 A new brain organoid model to study Parkinson’s DiseaseBolognin, Silvia  ; Smits, Lisa ; Nickels, Sarah Louise et alin Biomedical Science and Engineering (2021) Detailed reference viewed: 191 (24 UL)  Monitoring the neurotransmitter release of human midbrain organoids using a redox cycling microsensor as a novel tool for personalized Parkinson’s disease modeling and drug screening.; ; et al in Analyst (2021) Detailed reference viewed: 43 (0 UL)Integrated, automated maintenance, expansion and differentiation of 2D and 3D patient-derived cellular models for high throughput drug screeningBoussaad, Ibrahim ; Cruciani, Gérald ; Bolognin, Silvia et alin Scientific Reports (2021) Detailed reference viewed: 146 (5 UL) A Triple Combination of Targeting Ligands Increases the Penetration of Nanoparticles across a Blood-Brain Barrier Culture Model; ; et al in Pharmaceutics (2021) Detailed reference viewed: 44 (0 UL)The Parkinson’s-disease-associated mutation LRRK2-G2019S alters dopaminergic differentiation dynamics via NR2F1; Bolognin, Silvia ; et alin Cell Reports (2021) Detailed reference viewed: 91 (10 UL)Parkinson’s disease phenotypes in patient neuronal cultures and brain organoids improved by 2-Hydroxypropyl-b-Cyclodextrin treatmentJarazo, Javier ; Barmpa, Kyriaki ; et alin Movement Disorders (2021) Detailed reference viewed: 129 (24 UL)Structural Plasticity of Dopaminergic Neurons Requires the Activation of the D3R-nAChR Heteromer and the PI3K-ERK1/2/Akt-Induced Expression of c-Fos and p70S6K Signaling Pathway; ; et al in Molecular Neurobiology (2021) Detailed reference viewed: 34 (0 UL)A patient-based model of RNA mis-splicing uncovers treatment targets in Parkinson's disease.Boussaad, Ibrahim ; ; et alin Science translational medicine (2020), 12(560), Parkinson's disease (PD) is a heterogeneous neurodegenerative disorder with monogenic forms representing prototypes of the underlying molecular pathology and reproducing to variable degrees the sporadic ... [more ▼] Parkinson's disease (PD) is a heterogeneous neurodegenerative disorder with monogenic forms representing prototypes of the underlying molecular pathology and reproducing to variable degrees the sporadic forms of the disease. Using a patient-based in vitro model of PARK7-linked PD, we identified a U1-dependent splicing defect causing a drastic reduction in DJ-1 protein and, consequently, mitochondrial dysfunction. Targeting defective exon skipping with genetically engineered U1-snRNA recovered DJ-1 protein expression in neuronal precursor cells and differentiated neurons. After prioritization of candidate drugs, we identified and validated a combinatorial treatment with the small-molecule compounds rectifier of aberrant splicing (RECTAS) and phenylbutyric acid, which restored DJ-1 protein and mitochondrial dysfunction in patient-derived fibroblasts as well as dopaminergic neuronal cell loss in mutant midbrain organoids. Our analysis of a large number of exomes revealed that U1 splice-site mutations were enriched in sporadic PD patients. Therefore, our study suggests an alternative strategy to restore cellular abnormalities in in vitro models of PD and provides a proof of concept for neuroprotection based on precision medicine strategies in PD. [less ▲] Detailed reference viewed: 226 (29 UL)Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid model of CLN3-juvenile neuronal ceroid lipofuscinosisGomez Giro, Gemma ; ; Jarazo, Javier et alin Acta Neuropathologica Communications (2020) Detailed reference viewed: 209 (31 UL)Machine learning-assisted neurotoxicity prediction in human midbrain organoidsMonzel, Anna Sophia ; ; Smits, Lisa et alin Parkinsonism and Related Disorders (2020) Detailed reference viewed: 128 (21 UL)Impaired dopamine D3 and nicotinic acetylcholine receptor membrane localization in iPSCs-derived dopaminergic neurons from two Parkinson’s disease patients carrying the LRRK2 G2019S mutation; ; et al in Neurobiology of Aging (2020) Detailed reference viewed: 47 (0 UL)Reduced astrocytic reactivity in human brains and midbrain organoids with PRKN mutations; ; et al in NPJ Parkinson's Disease (2020) Detailed reference viewed: 53 (2 UL)Epitope imprinting of alpha-synuclein for sensing in Parkinson's brain organoid culture medium; ; et al in Biosensors and Bioelectronics (2020) Detailed reference viewed: 65 (0 UL)Single-cell transcriptomics reveals multiple neuronal cell types in human midbrain-specific organoidsSmits, Lisa ; Magni, Stefano ; et alin Cell and Tissue Research (2020) Detailed reference viewed: 114 (14 UL)Peptide-Imprinted Poly(hydroxymethyl 3,4-ethylenedioxythiophene) Nanotubes for Detection of Alpha Synuclein in Human Brain Organoids; ; et al in ACS Applied Nano Materials (2020) Detailed reference viewed: 108 (5 UL)Cultivation and characterization of human midbrain organoids in sensor integrated microfluidic chips; ; Bolognin, Silvia et alE-print/Working paper (2019) Detailed reference viewed: 95 (4 UL)Single-cell transcriptomics reveals multiple neuronal cell types in human midbrain-specific organoidsSmits, Lisa ; Magni, Stefano ; Grzyb, Kamil et alE-print/Working paper (2019) Human stem cell-derived organoids have great potential for modelling physiological and pathological processes. They recapitulate in vitro the organisation and function of a respective organ or part of an ... [more ▼] Human stem cell-derived organoids have great potential for modelling physiological and pathological processes. They recapitulate in vitro the organisation and function of a respective organ or part of an organ. Human midbrain organoids (hMOs) have been described to contain midbrain-specific dopaminergic neurons that release the neurotransmitter dopamine. However, the human midbrain contains also additional neuronal cell types, which are functionally interacting with each other. Here, we analysed hMOs at high-resolution by means of single-cell RNA-sequencing (scRNA-seq), imaging and electrophysiology to unravel cell heterogeneity. Our findings demonstrate that hMOs show essential neuronal functional properties as spontaneous electrophysiological activity of different neuronal subtypes, including dopaminergic, GABAergic, and glutamatergic neurons. Recapitulating these in vivo features makes hMOs an excellent tool for in vitro disease phenotyping and drug discovery. [less ▲] Detailed reference viewed: 298 (53 UL)Modeling Parkinson’s disease in midbrain-like organoidsSmits, Lisa ; ; et alin NPJ Parkinson's Disease (2019) Detailed reference viewed: 293 (24 UL)Impaired serine metabolism complements LRRK2-G2019S pathogenicity in PD patientsNickels, Sarah ; ; Bolognin, Silvia et alin Parkinsonism and Related Disorders (2019) Detailed reference viewed: 257 (43 UL)Neural Stem Cells of Parkinson's Disease Patients Exhibit Aberrant Mitochondrial Morphology and Functionality; Bolognin, Silvia ; Antony, Paul et alin Stem Cell Reports (2019) Detailed reference viewed: 308 (37 UL)
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