Search Results - "Glass, Hannes"
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Cell-Type-Dependent Recruitment Dynamics of FUS Protein at Laser-Induced DNA Damage Sites
Published in International journal of molecular sciences (01-03-2024)“…Increased signs of DNA damage have been associated to aging and neurodegenerative diseases. DNA damage repair mechanisms are tightly regulated and involve…”
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Pathomechanisms of ALS8: altered autophagy and defective RNA binding protein (RBP) homeostasis due to the VAPB P56S mutation
Published in Cell death & disease (10-05-2021)“…Mutations in RNA binding proteins (RBPs) and in genes regulating autophagy are frequent causes of familial amyotrophic lateral sclerosis (fALS). The P56S…”
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Human Spinal Motor Neurons Are Particularly Vulnerable to Cerebrospinal Fluid of Amyotrophic Lateral Sclerosis Patients
Published in International journal of molecular sciences (18-05-2020)“…Amyotrophic lateral sclerosis (ALS) is the most common and devastating motor neuron (MN) disease. Its pathophysiological cascade is still enigmatic. More than…”
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Mitochondria-Endoplasmic Reticulum Contact Sites Dynamics and Calcium Homeostasis Are Differentially Disrupted in PINK1-PD or PRKN-PD Neurons
Published in Movement disorders (01-10-2023)“…It is generally believed that the pathogenesis of PINK1/parkin-related Parkinson's disease (PD) is due to a disturbance in mitochondrial quality control…”
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Combined Dendritic and Axonal Deterioration Are Responsible for Motoneuronopathy in Patient-Derived Neuronal Cell Models of Chorea-Acanthocytosis
Published in International journal of molecular sciences (05-03-2020)“…Chorea acanthocytosis (ChAc), an ultra-rare devastating neurodegenerative disease, is caused by mutations in the gene, which encodes for the protein chorein…”
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Defective mitochondrial and lysosomal trafficking in chorea-acanthocytosis is independent of Src-kinase signaling
Published in Molecular and cellular neuroscience (01-10-2018)“…Mutations in the VPS13A gene leading to depletion of chorein protein are causative for Chorea Acanthocytosis (ChAc), a rare devastating disease, which is…”
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Changes in Blood Cell Deformability in Chorea-Acanthocytosis and Effects of Treatment With Dasatinib or Lithium
Published in Frontiers in physiology (04-04-2022)“…Misshaped red blood cells (RBCs), characterized by thorn-like protrusions known as acanthocytes, are a key diagnostic feature in Chorea-Acanthocytosis (ChAc),…”
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Corrosion Products from Metallic Implants Induce ROS and Cell Death in Human Motoneurons In Vitro
Published in Journal of functional biomaterials (25-07-2023)“…Due to advances in surgical procedures and the biocompatibility of materials used in total joint replacement, more and younger patients are undergoing these…”
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Applied Bayesian Approaches for Research in Motor Neuron Disease
Published in Frontiers in neurology (24-03-2022)“…Statistical evaluation of empirical data is the basis of the modern scientific method. Available tools include various hypothesis tests for specific data…”
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The Erythrocyte Sedimentation Rate and Its Relation to Cell Shape and Rigidity of Red Blood Cells from Chorea-Acanthocytosis Patients in an Off-Label Treatment with Dasatinib
Published in Biomolecules (Basel, Switzerland) (12-05-2021)“…Background: Chorea-acanthocytosis (ChAc) is a rare hereditary neurodegenerative disease with deformed red blood cells (RBCs), so-called acanthocytes, as a…”
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High content organelle trafficking enables disease state profiling as powerful tool for disease modelling
Published in Scientific data (13-11-2018)“…Neurodegenerative diseases pose a complex field with various neuronal subtypes and distinct differentially affected intra-neuronal compartments. Modelling of…”
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Functional and Molecular Properties of DYT-SGCE Myoclonus-Dystonia Patient-Derived Striatal Medium Spiny Neurons
Published in International journal of molecular sciences (30-03-2021)“…Myoclonus-dystonia (DYT-SGCE, formerly DYT11) is characterized by alcohol-sensitive, myoclonic-like appearance of fast dystonic movements. It is caused by…”
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A serum microRNA sequence reveals fragile X protein pathology in amyotrophic lateral sclerosis
Published in Brain (London, England : 1878) (07-05-2021)“…Knowledge about converging disease mechanisms in the heterogeneous syndrome amyotrophic lateral sclerosis (ALS) is rare, but may lead to therapies effective in…”
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Knocking out C9ORF72 Exacerbates Axonal Trafficking Defects Associated with Hexanucleotide Repeat Expansion and Reduces Levels of Heat Shock Proteins
Published in Stem cell reports (10-03-2020)“…In amyotrophic lateral sclerosis (ALS) motor neurons (MNs) undergo dying-back, where the distal axon degenerates before the soma. The hexanucleotide repeat…”
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Restoring Axonal Organelle Motility and Regeneration in Cultured FUS-ALS Motoneurons through Magnetic Field Stimulation Suggests an Alternative Therapeutic Approach
Published in Cells (Basel, Switzerland) (29-05-2023)“…Amyotrophic lateral sclerosis (ALS) is a devastating motoneuron disease characterized by sustained loss of neuromuscular junctions, degenerating corticospinal…”
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Therapeutic targeting of Lyn kinase to treat chorea-acanthocytosis
Published in Acta neuropathologica communications (03-05-2021)“…Chorea-Acanthocytosis (ChAc) is a devastating, little understood, and currently untreatable neurodegenerative disease caused by VPS13A mutations. Based on our…”
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Differentiation Efficiency of Induced Pluripotent Stem Cells Depends on the Number of Reprogramming Factors
Published in Stem cells (Dayton, Ohio) (01-03-2012)“…Reprogramming of somatic cells into induced pluripotent stem cells (iPSCs) by retroviral overexpression of the transcription factors Oct4, Sox2, Klf4, and…”
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Glycolic acid and D-lactate-putative products of DJ-1-restore neurodegeneration in FUS - and SOD1-ALS
Published in Life science alliance (01-08-2024)“…Amyotrophic lateral sclerosis (ALS) leads to death within 2-5 yr. Currently, available drugs only slightly prolong survival. We present novel insights into the…”
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Live Cell Imaging of ATP Levels Reveals Metabolic Compartmentalization within Motoneurons and Early Metabolic Changes in FUS ALS Motoneurons
Published in Cells (Basel, Switzerland) (09-05-2023)“…Motoneurons are one of the most energy-demanding cell types and a primary target in Amyotrophic lateral sclerosis (ALS), a debilitating and lethal…”
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