Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines

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  1. Excitoxicity and excitatory amino acid antagonists in chronic neurodegenerative diseases
    Chrysanthy Ikonomidou, Lechosław Turski · 2005 · Cambridge University Press eBooks
  2. Huntingtin toxicity is ameliorated by BAG1 through modulation of its aggregation, degradation and subcellular distribution
    Kamila Sroka · 2009 · eDiss (Georg-August-Universität Göttingen)
  3. Mitochondrial trafficking in healthy and injured neurons
    Diane T. W. Chang · 2005 · D-Scholarship@Pitt (University of Pittsburgh)
  4. The Role of Mitochondrial Dysfunction in Neurodegenerative Proteinopathies and Aging.
    Alejandro Ocampo · 2012
  5. Testing of neuroprotective compounds in a transgenic mouse model of Huntington's disease
    Dénes Zádori · 2011
  6. A role for neuroglobin in the inhibition of cytochrome c-mediated apoptosome formation
    Kristen Henty · 2011 · ResearchSpace (University of Auckland)
  7. The burst of mitochondrial diseases: neurons and calcium.
    Anna Fendyur, Igor Kaiserman, Lior M. Kasinetz, Rami Rahamimoff · 2004 · PubMed
  8. Neuropathological characterization of FL-PGC-1α-deficient mice – implications for mitochondrial encephalopathy
    Levente Szalárdy · 2016 · SZTE Repository of Dissertations (University of Szeged)
  9. Ribosome Profiling Reveals a Dichotomy Between Ribosome Occupancy of Nuclear-Encoded and Mitochondrial-Encoded OXPHOS mRNA Transcripts in a Striatal Cell Model of Huntington Disease
    Srinivasa Subramaniam, Neelam Shahani · 2021 · bioRxiv (Cold Spring Harbor Laboratory)
  10. Huntingtin aggregation at interfaces associated with membranes and organelles
    Adewale Adegbuyiro · 2021
  11. Picosecond Pulsed Electric Field-Induced Disaggregation of Polyglutamine Aggregates in Huntington’s Disease Neural Stem Cells
    Mackenzie Tardif-Kunk, Martina Zamponi, Emily Old, Augustine U. Anthony, Chandler Knox, Thomas R. Campbell, Ross A. Petrella, Patrick C. Sachs, Robert D. Bruno, Peter A. Mollica · 2025 · Research Square
  12. Molecular Pathogenesis of Huntington's Disease: The Role of Excitotoxicity
    Mahmoud A. Pouladi, Безпрозванный Илья Борисович, Lynn A. Raymond, Michael R. Hayden · 2006 · Elsevier eBooks
  13. Glutamate, Calcium and Neurodegenerative Disease: Impact of Cytosolic Calcium Buffers and Their Potential Role for Neuroprotective Strategies
    Friederike Bergmann, Bernhard U. Keller · 2004 · Springer eBooks
  14. Desenvolvimento de um modelo in vitro dos efeitos citotóxicos da ataxina-3 expandida e avaliação de diferentes estratégias terapêuticas para o controle desses efeitos/
    Camila Miranda Lopes · 2010
  15. The molecular pathogenesis of Huntington’s disease
    Christopher D. Turner · 2009 · UCL Discovery (University College London)
  16. The Study of Two Strategies for Decreasing Mutant Huntingtin: Degradation by Puromycin Sensitive AminoPeptidase and RNA Interference: A Dissertation
    Joanna Chaurette · 2013
  17. THE ROLE OF P53 IN OXIDATIVE STRESS AND POLYGLUTAMINE NEUROTOXICITY
    Jay C. Dunn · 2003 · UKnowledge (University of Kentucky)
  18. Etiology, Pathology, and Pathogenesis
    Patrick Weydt, G. Bernhard Landwehrmeyer, Albert Christian Ludolph · 2009 · Blue books of neurology
  19. Cellular Toxicity of Protein Aggregates
    Bruce L. Kagan · 2008 · Wiley eBooks
  20. Towards a Novel Treatment of Huntington´s Disease
    Mouna Esmaeilzadeh · 2011 · Figshare
  21. Ameliorating Huntington's Disease by Targeting Huntingtin mRNA
    Mackenzie Melvin, Rinkse Vlamings, Yasin Temel, Willeke van Roon‐Mom · 2012 · InTech eBooks
  22. Application of a Drosophila model to study Huntington's disease pathogenesis in vivo
    László Bodai · 2004 · SZTE Repository of Dissertations (University of Szeged)
  23. Redox Regulation of Protein Misfolding, Synaptic Damage, and Neuronal Loss in Neurodegenerative Diseases
    Tomohiro Nakamura, Stuart A. Lipton · 2011 · Wiley eBooks
  24. Huntington's disease modeling and treatment : from primary neuronal cultures to rodents
    Diana Zala · 2005 · Infoscience (Ecole Polytechnique Fédérale de Lausanne)
  25. β-cell dysfunction in Huntington's disease
    Karl Bacos · 2009 · Lund University Publications (Lund University)
  26. Beyond the basal ganglia
    Michael Burg, m Jorien · 2008 · Lund University Publications (Lund University)
  27. Copy Number Variation, Eicosapentaenoic Acid and Neurological Disorders
    Basant K. Puri, Mehar S. Manku · 2010 · Lifestyle Genomics
  28. B10 The role of n-terminal phosphorylation in huntingtin's oligomerisation, aggregation and toxicity
    Federico Herrera, J. Branco dos Santos, Tiago F. Outeiro · 2012 · Journal of Neurology Neurosurgery & Psychiatry
  29. Molecular biology of the InsP3Rs: focus on brain function in health and disease
    Chihiro Hisatsune, Katsuhiko Mikoshiba · 2012 · Wiley Interdisciplinary Reviews Membrane Transport and Signalling
  30. Malattia di Huntington: aspetti diagnostici attuali e applicazioni pratiche
    Katia Youssov, Anne‐Catherine Bachoud‐Lévi · 2008 · EMC - Neurologia
  31. Molecular Pathways of Mitochondrial Dysfunction in Neurodegeneration: the Paradigms of Parkinson's and Huntington's Diseases
    Ana Cristina Rego, Sandra M. Cardoso, Catarina Resende Oliveira · 2007 · Springer eBooks
  32. A Genome-Wide RNAi Screen for Modifiers of Polyglutamine-Induced Neurotoxicity in Drosophila
    Hannes Voßfeldt · 2013 · eDiss (Georg-August-Universität Göttingen)
  33. Murine Models of Huntington Disease
    Aaron Sturrock, Blair R. Leavitt · 2009 · Future Neurology
  34. Creation and characterization of mitochondrial dna-depleted human huntington’s disease and control derived lymphoblasts
    Ribeiro, Maria João Rodrigues Ferreira · 2010 · Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT)
  35. IN HEALTHY AND INJURED NEURONS
    Diane T. W. Chang · 2005
  36. Organelle specific mechanisms of neuronal cell death
    Noora Putkonen · 2012 · Työväentutkimus Vuosikirja
  37. Monitoring transcriptomic responses of SH-SY5Y cells to amyloid treatments
    Abhinav K. Jain · 2025 · Library, Museums and Press - UDSpace (University of Delaware)
  38. Mitochondria, metabolic inhibitors and neurodegeneration
    James G. Greene, J. Timothy Greenamyre · 2005 · Cambridge University Press eBooks
  39. Polyglutamine Diseases
    H. Okazawa · 2007 · Springer eBooks
  40. Maladies par expansion de polyglutamine: Avancées récentes et perspectives thérapeutiques Polyglutamine expansion diseases: Recent advances and therapeutic perspectives Revue de la littérature
    Gretta Abou Sleymane, Yvon Trottier · 2010 · HAL
  41. Modelling and treating Huntington’s disease: Generation of high-capacity adenovirus vectors to express normal and mutant huntingtin and to block huntingtin expression by short hairpin interference RNA
    Bin Huang · 2008 · OPen Access Repositorium der Universität Ulm (OPARU) (Ulm University)
  42. Huntington's Disease: Unraveling the Pathophysiological Cascade Behind a Singular Gene Defect
    Christoph Michael Kosinski, G. Bernhard Landwehrmeyer, Andrea G. Ludolph · 2007 · Springer eBooks
  43. Role of Mitochondria in Parkinson’s Disease and Huntington’s Disease
    Anthony H.V. Schapira · 2012 · Oxidative stress and disease
  44. Huntington’s Disease
    Lynn A. Raymond, Blair R. Leavitt · 2005 · Neurological disease and therapy
  45. Clinical and Pathological Characteristics of Huntington Disease
    Jayaraman Rao · 2005 · Elsevier eBooks
  46. UNIVERSIDADE FEDERAL DO CEARÁ FACULDADE DE MEDICINA DEPARTAMENTO DE FISIOLOGIA E FARMACOLOGIA PROGRAMA DE PÓS-GRADUAÇÃO EM FARMACOLOGIA
    Maria Carvalho Moura · 2012
  47. Stem cell therapies for Huntington's disease
    Andrew T. Crane, Julien Rossignol, Gary Leo Dunbar · 2016 · Wiley eBooks
  48. The effect of mitochondrial toxins on cytochrome c oxidase subunit IV isoforms and its consequences on mitochondrial function and neural cell survival
    Magdalena Misiak, Susanne Arnold · 2010 · RWTH Publications (RWTH Aachen)
  49. Defective Dynamics Of Mitochondria In Amyotrophic Lateral Sclerosis And Huntington's Disease
    Wenjun Song · 2012 · Journal of International Crisis and Risk Communication Research
  50. Identification and Biophysical Characterization of Small Molecules Modulating Protein Disulfide Isomerase in Neurodegenerative Diseases
    Anna R. Kaplan · 2015
  51. Cytochrome c oxidase mediates the regulation of mitochondrial function in an in vitro model of Huntington's disease
    Shilpee Singh, H. N. Wagner · 2009 · RWTH Publications (RWTH Aachen)
  52. Molecular mechanisms underlying selective clearance of protein aggregates and mitochondria by autophagy
    Sijie Tan · 2017 · DR-NTU (Nanyang Technological University)
  53. Searching for Correlations Between the Development of Neurodegenerative Hallmarks: Targeting Huntingtin as a Contributing Factor
    Nelina P. Angelova · 2017 · Advances in experimental medicine and biology
  54. Measurement of Oxygen MetabolismIn Vivo
    Marcus P.J. van Diemen, Rinse Ubbink, F. Michael Münker, Egbert Gezinus Mik, Geert Jan Groeneveld · 2018 · Wiley eBooks
  55. A Transgenic Monkey Model of Huntington’s Disease
    In Ki Cho, Anthony W.S. Chan · 2018 · Elsevier eBooks
  56. Huntington’s Disease and Other Polyglutamine Repeat Diseases
    Audrey S. Dickey, Albert R. La Spada · 2018 · Elsevier eBooks
  57. Role of autophagy in GM1-mediated neuroprotection in Huntington's Disease
    Sabine Schmelz · 2016 · ERA: Education and Research Archive (University of Alberta)
  58. Understanding Huntington's disease using Machine Learning Approaches
    Sonali Lokhande · 2017 · Scholarship - Claremont (Claremont Colleges)
  59. Neurodegenerative Disorders Progression
    Ramneek Kaur, Harleen Kaur, Rashi Rajput, Sachin Kumar, R. Rachana, Manisha Singh · 2018 · Advances in medical diagnosis, treatment, and care (AMDTC) book series
  60. IP3 Receptor and Ca2+ Signaling
    Chihiro Hisatsune, Katsuhiko Mikoshiba · 2009 · Springer eBooks
  61. Modulation of neuronal mitochondrial dynamics, autophagy and huntingtin proteostasis by HDAC inhibitors: Insights for Huntington's disease
    Pedro Gonçalo Guedes Dias · 2015 · Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT)
  62. Pathways of neurodegeneration underlying dementia
    Noel J. Buckley, George K. Tofaris · 2020
  63. Calcium Dysregulation in Neurodegenerative Diseases
    Haroon Anwar · 2018 · Springer eBooks
  64. The Transient Receptor Potential Vanilloid-1 Channel and Neuronal Survival in Degenerative Disease
    Nicholas J. Ward · 2014
  65. In vivo progressive degeneration of Huntington’s disease patient-derived neurons reveals human-specific pathological phenotypes
    Andrés Miguez, Sara Fernández‐García, Marta Tortajada, Georgina Bombau, Mireia Galofré, Marı́a Garcı́a-Bravo, Cristina Vila, Phil Sanders, Helena Fernández-Medina, Blanca Poquet et al. · 2020 · bioRxiv (Cold Spring Harbor Laboratory)
  66. Protein/peptide characterization using mass spectrometry and molecular dynamics simulations
    Ahmad Kiani Karanji · 2020 · The Research Repository @ WVU (West Virginia University)
  67. The effects of membrane physicochemical properties on huntingtin membrane association and downstream aggregation
    Maryssa A. Beasley · 2020 · The Research Repository @ WVU (West Virginia University)
  68. Нормализация кальциевого баланса в нейронах стриатума при болезни хантингтона: роль сигма-1-рецептора как потенциальной мишени для терапии
    Н А Красковская, Безпрозванный Илья Борисович · 2021 · Biochemistry (Moscow)
  69. Factors Influencing Huntingtin Aggregation at Surfaces: Implications for Huntington’s Disease
    Sharon E. Groover · 2020
  70. Targeting the Nt17 of the huntingtin protein via natural and chemical modifications: impact on aggregation and membrane interactions
    Faezeh Sedighi · 2020
  71. RNA-Mediated Metabolic Defects in Microsatellite Expansion Diseases
    Nan Zhang · 2021 · Springer eBooks
  72. Resveratrol and brain mitochondria
    Patrícia Coelho, Ana Cristina Rego · 2021 · Elsevier eBooks
  73. Polyglutamine Diseases
    Meyer J. Friedman, Shi-Hua Li, Xiao‐Jiang Li · 2007 · Springer eBooks
  74. Transglutaminase
    Arthur J.L. Cooper, S.-Y. Kim · 2007 · Springer eBooks
  75. The paradigm of Huntington’s disease: Therapeutic opportunities in neurodegeneration
    Julie Leegwater‐Kim, Jang-Ho J. Cha · 2004 · Neurotherapeutics
  76. Mitochondria-targeted drug delivery systems for the effective treatment of neurodegenerative disorders
    Vaishali Khare, Surbhi Gupta, Preeti Bisht, Debapriya Garabadu · 2022 · Elsevier eBooks
  77. P.570 Effects of ginseng and ginsenosides on mutant huntingtin aggregation, cellular apoptosis, mitochondrial dysfunction and motor impairment of Huntington's disease model
    J.J. Ban, W. Im · 2019 · European Neuropsychopharmacology
  78. Faculty Opinions recommendation of PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function.
    X William Yang, Daniel C. Lee · 2012 · Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature
  79. Faculty Opinions recommendation of Inhibition of mitochondrial protein import by mutant huntingtin.
    Stefano Di Donato · 2014 · Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature
  80. Malattia di Huntington: aspetti diagnostici attuali e applicazioni pratiche
    Katia Youssov, Anne‐Catherine Bachoud‐Lévi · 2018 · EMC - Neurologia
  81. Calcium Dysregulation in Neurodegenerative Diseases
    Haroon Anwar · 2022 · Springer eBooks
  82. Neurotoxicity in Huntington Disease
    Clement T. Loy, Anthony John Hannan · 2022 · Springer eBooks
  83. Exogenous factors that impact huntingtin aggregation
    Adam Skeens · 2023 · The Research Repository @ WVU (West Virginia University)
  84. Lipid binding properties of huntingtin as a novel therapeutic target
    Chathuranga Siriwardhana · 2023
  85. Investigation of Early Complex Formation of Huntingtin Protein With and Without Lipids
    Alyssa R. Stonebraker · 2023 · The Research Repository @ WVU (West Virginia University)
  86. Mitochondrial Ca2+ Handling in the Central Nervous System: the role of the mitochondrial Na+>/sup sup<2+ exchanger (NCLX) in astrocyte metabolism and function
    João Victor Cabral Costa · 2022 · Digital Library of Theses and Dissertations (Universidade de São Paulo)
  87. Buccal Respiratory Chain Complexes I and IV Quantities in Huntington’s Disease Patients
    Petr Dušek, Marie Rodinová, I. Lišková, Jiří Klempíř, J Zeman, Jan Roth, Hana Hansíková · 2018 · Folia Biologica
  88. Güncel Biyokimya Çalışmaları VII
    Yasemin Atıcı, Zeynep Ökten, Sara Kasebi, Buse Semerci Arıkan, Mehmet Erdem, Tuğba Raika Kıran, Abdullah Kaan Yağız, Berat Aktolun, Yeşim Güvenç Demirağcı, Hatibe Kara et al. · 2024 · Akademisyen Kitabevi eBooks
  89. Protein Metabolism: Critical Factors Implicated in Neurodegenerative Diseases
    Jyoti Singh, Bappi Sarkar, Ashim Mukherjee, Mousumi Mutsuddi · 2024 · Springer eBooks
  90. The role of mitochondria in pathogenesis of Huntington's disease
    M Marková, Hana Hansíková · 2016 · Klinická biochemie a metabolismus
  91. High Content System for Quantifying Mitochondrial Morphology in Patient-Derived Human Cells
    Kaitlyn Deschamps, Samantha R Laville-Dupuy, Christina Yi Peng, Ray Truant · 2025 · bioRxiv (Cold Spring Harbor Laboratory)
  92. Creatina e seus benefícios preventivos contra doenças cognitivas e neurodegenerativas
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  93. DPV UL38 stabilizes MFN2 to subvert MAVS-mediated antiviral immunity in ducks
    Bin Tian, Xuetong Wang, Shuyi He, Dongjie Cai, Yanming Tian, Mingshu Wang, Renyong Jia, Shun Chen, Dekang Zhu, Mafeng Liu et al. · 2025 · Veterinary Microbiology
  94. Clinical, laboratory, and genetic studies of families with Charcot-Marie-Tooth type 2C disease
    Guida Landouré · 2011 · UCL Discovery (University College London)
  95. Excitotoxicidade em células granulares do cerebelo que expressam ataxina-3 mutante - relevância para a patogénese da doença de Machado-Joseph
    Sofia Isabel Oliveira Sousa · 2011 · Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT)
  96. The clinical utility of multidisciplinary rehabilitation in individuals with Huntington’s Disease
    Travis M. Cruickshank · 2015 · Australasian Journal of Paramedicine
  97. Additional file 1 of Neuronal cell-based high-throughput screen for enhancers of mitochondrial function reveals luteolin as a modulator of mitochondria-endoplasmic reticulum coupling
    Luana Naia, Catarina Moreira Pinho, Giacomo Dentoni, Jianping Liu, Leal, Nuno Santos, Duarte M. S. Ferreira, Bernadette Schreiner, Riccardo Filadi, Lígia Fão, Niamh M. C. Connolly et al. · 2021 · Figshare
  98. Additional file 1 of Disruption of the mitochondrial network in a mouse model of Huntington's disease visualized by in-tissue multiscale 3D electron microscopy
    Eva Martin‐Solana, Casado-Zueras, Laura, Teobaldo Enrique Torres, Gerardo Fabián Goya, Fernandez-Fernandez, Maria-Rosario, Fernandez, Jose-Jesus · 2024 · Figshare
  99. Activation of TPC2 amplifies lysosome-mitochondria calcium transfer to regulate energetic stress responses
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  100. Étude de l'implication potentielle des marqueurs du striatum dans la maladie de Huntington
    Laurie Galvan · 2011 · theses.fr (ABES)