Showing 1 - 20 results of 40 for search '"neurodegeneration"', query time: 0.08s Refine Results
  1. 1
    FUS ALS neurons activate major stress pathways and reduce translation as an early protective mechanism against neurodegeneration

    FUS ALS neurons activate major stress pathways and reduce translation as an early protective mechanism against neurodegeneration by Barbara Szewczyk, René Günther, Julia Japtok, Moritz J. Frech, Marcel Naumann, Hyun O. Lee, Andreas Hermann

    Published 2023-02-01
    “…While inhibition of SG formation, translation, or ISR does not influence survival of FUS ALS neurons, proteotoxicity that cannot be compensated with the activation of stress pathways is the main driver of neurodegeneration in early FUS ALS.…”
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  2. 2
    Commentary: Acanthocytes identified in Huntington's disease

    Commentary: Acanthocytes identified in Huntington's disease by Kevin Peikert, Kevin Peikert, Kevin Peikert, Alexander Storch, Alexander Storch, Alexander Storch, Andreas Hermann, Andreas Hermann, Andreas Hermann, G. Bernhard Landwehrmeyer, Ruth H. Walker, Ruth H. Walker, Greta Simionato, Greta Simionato, Lars Kaestner, Lars Kaestner, Adrian Danek, Adrian Danek

    Published 2022-11-01
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  3. 3
    Interleukin-17 and Th17 Lymphocytes Directly Impair Motoneuron Survival of Wildtype and FUS-ALS Mutant Human iPSCs

    Interleukin-17 and Th17 Lymphocytes Directly Impair Motoneuron Survival of Wildtype and FUS-ALS Mutant Human iPSCs by Mengmeng Jin, Katja Akgün, Tjalf Ziemssen, Markus Kipp, Rene Günther, Andreas Hermann

    Published 2021-07-01
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  4. 4
    Chronic–Progressive Dopaminergic Deficiency Does Not Induce Midbrain Neurogenesis

    Chronic–Progressive Dopaminergic Deficiency Does Not Induce Midbrain Neurogenesis by Mareike Fauser, Francisco Pan-Montojo, Christian Richter, Philipp J. Kahle, Sigrid C. Schwarz, Johannes Schwarz, Alexander Storch, Andreas Hermann

    Published 2021-03-01
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  5. 5
    Oxidative Stress and Alterations in the Antioxidative Defense System in Neuronal Cells Derived from NPC1 Patient-Specific Induced Pluripotent Stem Cells

    Oxidative Stress and Alterations in the Antioxidative Defense System in Neuronal Cells Derived from NPC1 Patient-Specific Induced Pluripotent Stem Cells by Alexandra V. Jürs, Christin Völkner, Maik Liedtke, Katharina Huth, Jan Lukas, Andreas Hermann, Moritz J. Frech

    Published 2020-10-01
    “…Consequently, OS represents a common pathophysiological hallmark of neurodegenerative diseases and is discussed to contribute to the neurodegeneration observed amongst others in Alzheimer’s disease and Parkinson’s disease. …”
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  6. 6
    Direct Interaction of ATP7B and LC3B Proteins Suggests a Cooperative Role of Copper Transportation and Autophagy

    Direct Interaction of ATP7B and LC3B Proteins Suggests a Cooperative Role of Copper Transportation and Autophagy by Supansa Pantoom, Adam Pomorski, Katharina Huth, Christina Hund, Janine Petters, Artur Krężel, Andreas Hermann, Jan Lukas

    Published 2021-11-01
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  7. 7
    Assessment of FDA-Approved Drugs as a Therapeutic Approach for Niemann-Pick Disease Type C1 Using Patient-Specific iPSC-Based Model Systems

    Assessment of FDA-Approved Drugs as a Therapeutic Approach for Niemann-Pick Disease Type C1 Using Patient-Specific iPSC-Based Model Systems by Christin Völkner, Supansa Pantoom, Maik Liedtke, Jan Lukas, Andreas Hermann, Moritz J. Frech

    Published 2022-01-01
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  8. 8
    Pathophysiological In Vitro Profile of Neuronal Differentiated Cells Derived from Niemann-Pick Disease Type C2 Patient-Specific iPSCs Carrying the <i>NPC2</i> Mutations c.58G>T/c.140G>T

    Pathophysiological In Vitro Profile of Neuronal Differentiated Cells Derived from Niemann-Pick Disease Type C2 Patient-Specific iPSCs Carrying the <i>NPC2</i> Mutations c.58G>T/c.1... by Maik Liedtke, Christin Völkner, Alexandra V. Jürs, Franziska Peter, Michael Rabenstein, Andreas Hermann, Moritz J. Frech

    Published 2021-04-01
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  9. 9
    Cell-Type-Dependent Recruitment Dynamics of FUS Protein at Laser-Induced DNA Damage Sites

    Cell-Type-Dependent Recruitment Dynamics of FUS Protein at Laser-Induced DNA Damage Sites by Yu Niu, Arun Pal, Barbara Szewczyk, Julia Japtok, Marcel Naumann, Hannes Glaß, Andreas Hermann

    Published 2024-03-01
    “…FUS is a ubiquitously expressed protein, but if mutated, leads to a more or less selective motor neurodegeneration, causing amyotrophic lateral sclerosis (ALS). …”
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  10. 10
    Impact of Organelle Transport Deficits on Mitophagy and Autophagy in Niemann–Pick Disease Type C

    Impact of Organelle Transport Deficits on Mitophagy and Autophagy in Niemann–Pick Disease Type C by Maik Liedtke, Christin Völkner, Andreas Hermann, Moritz J. Frech

    Published 2022-02-01
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  11. 11
    Pluripotent Stem Cells for Disease Modeling and Drug Discovery in Niemann-Pick Type C1

    Pluripotent Stem Cells for Disease Modeling and Drug Discovery in Niemann-Pick Type C1 by Christin Völkner, Maik Liedtke, Andreas Hermann, Moritz J. Frech

    Published 2021-01-01
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  12. 12
    Generation of the human iPSC lines AKOSi011-A carrying the mutation p.Pro65Ser/p.Asp35T and AKOSi012-A, carrying the mutation p.Tyr231His, derived from FAHN patient fibroblasts

    Generation of the human iPSC lines AKOSi011-A carrying the mutation p.Pro65Ser/p.Asp35T and AKOSi012-A, carrying the mutation p.Tyr231His, derived from FAHN patient fibroblasts by Fatima Efendic, Saskia Krohn, Hugo Murua Escobar, Sunita Venkateswaran, Steffany A.L. Bennett, Andreas Hermann, Moritz J. Frech

    Published 2023-09-01
    “…Fatty acid hydroxylase-associated neurodegeneration (FAHN) is a hereditary neurodegenerative disease caused by mutations in the FA2H gene. …”
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  13. 13
    Applied Bayesian Approaches for Research in Motor Neuron Disease

    Applied Bayesian Approaches for Research in Motor Neuron Disease by Anna G. M. Temp, Anna G. M. Temp, Anna G. M. Temp, Marcel Naumann, Andreas Hermann, Andreas Hermann, Andreas Hermann, Hannes Glaß

    Published 2022-03-01
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  14. 14
    Corrosion Products from Metallic Implants Induce ROS and Cell Death in Human Motoneurons <i>In Vitro</i>

    Corrosion Products from Metallic Implants Induce ROS and Cell Death in Human Motoneurons <i>In Vitro</i> by Hannes Glaß, Anika Jonitz-Heincke, Janine Petters, Jan Lukas, Rainer Bader, Andreas Hermann

    Published 2023-07-01
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  15. 15
    Systematic estimation of biological age of in vitro cell culture systems by an age-associated marker panel

    Systematic estimation of biological age of in vitro cell culture systems by an age-associated marker panel by Christiane Hartmann, Luise Herling, Alexander Hartmann, Verena Köckritz, Georg Fuellen, Georg Fuellen, Michael Walter, Michael Walter, Andreas Hermann, Andreas Hermann, Andreas Hermann

    Published 2023-02-01
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  16. 16
    Downstream Effects of Mutations in <i>SOD1</i> and <i>TARDBP</i> Converge on Gene Expression Impairment in Patient-Derived Motor Neurons

    Downstream Effects of Mutations in <i>SOD1</i> and <i>TARDBP</i> Converge on Gene Expression Impairment in Patient-Derived Motor Neurons by Banaja P. Dash, Axel Freischmidt, Jochen H. Weishaupt, Andreas Hermann

    Published 2022-08-01
    “…Despite extensive research, the reason for neurodegeneration is still not understood. To generate novel hypotheses of putative underlying molecular mechanisms, we used human induced pluripotent stem cell (hiPSCs)-derived motor neurons (MNs) from <i>SOD1</i>- and <i>TARDBP</i> (TDP-43 protein)-mutant-ALS patients and healthy controls to perform high-throughput RNA-sequencing (RNA-Seq). …”
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  17. 17
    Mechanistic Insight into the Mode of Action of Acid β-Glucosidase Enhancer Ambroxol

    Mechanistic Insight into the Mode of Action of Acid β-Glucosidase Enhancer Ambroxol by Supansa Pantoom, Larissa Hules, Christopher Schöll, Andranik Petrosyan, Maria Monticelli, Jola Pospech, Maria Vittoria Cubellis, Andreas Hermann, Jan Lukas

    Published 2022-03-01
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  18. 18
    Live Cell Imaging of ATP Levels Reveals Metabolic Compartmentalization within Motoneurons and Early Metabolic Changes in <i>FUS</i> ALS Motoneurons

    Live Cell Imaging of ATP Levels Reveals Metabolic Compartmentalization within Motoneurons and Early Metabolic Changes in <i>FUS</i> ALS Motoneurons by Vitaly L. Zimyanin, Anna-Maria Pielka, Hannes Glaß, Julia Japtok, Dajana Großmann, Melanie Martin, Andreas Deussen, Barbara Szewczyk, Chris Deppmann, Eli Zunder, Peter M. Andersen, Tobias M. Boeckers, Jared Sterneckert, Stefanie Redemann, Alexander Storch, Andreas Hermann

    Published 2023-05-01
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  19. 19
    Patient-Specific iPSC-Derived Neural Differentiated and Hepatocyte-like Cells, Carrying the Compound Heterozygous Mutation p.V1023Sfs*15/p.G992R, Present the “Variant” Biochemical Phenotype of Niemann-Pick Type C1 Disease

    Patient-Specific iPSC-Derived Neural Differentiated and Hepatocyte-like Cells, Carrying the Compound Heterozygous Mutation p.V1023Sfs*15/p.G992R, Present the “Variant” Biochemical... by Christin Völkner, Maik Liedtke, Robert Untucht, Andreas Hermann, Moritz J. Frech

    Published 2021-11-01
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  20. 20
    Adaptative Up-Regulation of PRX2 and PRX5 Expression Characterizes Brain from a Mouse Model of Chorea-Acanthocytosis

    Adaptative Up-Regulation of PRX2 and PRX5 Expression Characterizes Brain from a Mouse Model of Chorea-Acanthocytosis by Enrica Federti, Alessandro Matte, Veronica Riccardi, Kevin Peikert, Seth L. Alper, Adrian Danek, Ruth H. Walker, Angela Siciliano, Iana Iatcenko, Andreas Hermann, Lucia De Franceschi

    Published 2021-12-01
    “…Chorea-acanthocytosis/<i>Vps13A</i> disease (ChAc) is a devastating, life-shortening disorder characterized by acanthocytosis, neurodegeneration and abnormal proteostasis. We recently developed a <i>Vps13a<sup>−/−</sup></i> ChAc-mouse model, showing acanthocytosis, neurodegeneration and neuroinflammation which could be restored by LYN inactivation. …”
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