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

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  1. Genotype-, aging-dependent abnormal caspase activity in Huntington disease blood cells
    Ferdinando Squitieri, Vittorio Maglione, Sara Orobello, Francesco Fornai · 2011 · Journal of Neural Transmission
  2. DISC1 and Huntington's Disease – Overlapping Pathways of Vulnerability to Neurological Disorder?
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  3. Advances in Huntington Disease Drug Discovery: Novel Approaches to Model Disease Phenotypes
    Jonathan Bard, Michael D. Wall, Ovadia Lazari, Jamshid Arjomand, Ignacio Muñoz-Sanjuán · 2013 · SLAS DISCOVERY
  4. DNAzyme Cleavage of CAG Repeat RNA in Polyglutamine Diseases
    Nan Zhang, Brittani Bewick, Jason Schultz, Anjana Tiwari, Robert Krencik, Aijun Zhang, Kaho Adachi, Guangbin Xia, Kyuson Yun, Partha S. Sarkar et al. · 2021 · Neurotherapeutics
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  7. Common Variations in 4p Locus are Related to Male Completed Suicide
    Anne Must, Sulev Kõks, Eero Vasar, Gunnar Tasa, Aavo Lang, Eduard Maron, Marika M Väli · 2008 · NeuroMolecular Medicine
  8. Stem Cells Transplantation and Huntington’s Disease
    Wooseok Im, Soon‐Tae Lee, Kon Chu, Manho Kim, Jae‐Kyu Roh · 2009 · International Journal of Stem Cells
  9. RNA Aptamers Rescue Mitochondrial Dysfunction in a Yeast Model of Huntington’s Disease
    Kinjal A. Patel, Rajeev Kumar Chaudhary, Ipsita Roy · 2018 · Molecular Therapy — Nucleic Acids
  10. Do BDNF and NT‐4/5 exert synergistic or occlusive effects on corticostriatal transmission in a male mouse model of Huntington's disease?
    Francisco Miguel Torres-Cruz, Ernesto Mendoza, Israel César Vivar‐Cortés, Francisco García‐Sierra, Elizabeth Hernández · 2019 · Journal of Neuroscience Research
  11. Effects of Anthocyanins on CAG Repeat Instability and Behaviour in Huntington’s Disease R6/1 Mice
    Linda Møllersen, Olve Moldestad, Alexander D. Rowe, Anja Bjølgerud, Ingunn Holm, Linda Tveterås, Arne Klungland, Lars Retterstøl · 2016 · PLoS Currents
  12. Mutant huntingtin fails to directly impair brain mitochondria
    James Hamilton, Tatiana Brustovetsky, Nickolay Brustovetsky · 2019 · Journal of Neurochemistry
  13. Oxidation Promotes Distinct Huntingtin Aggregates in the Presence and Absence of Membranes
    Adewale Adegbuyiro, Alyssa R. Stonebraker, Faezeh Sedighi, Caleb K. Fan, Breanna L. Hodges, Peng Li, Stephen J. Valentine, Justin A. Legleiter · 2022 · Biochemistry
  14. Mitochondrial degradation of amyloidogenic proteins — A new perspective for neurodegenerative diseases
    Janin Lautenschläger, Gabriele S. Kaminski Schierle · 2019 · Progress in Neurobiology
  15. Macromolecular crowding in solution alters huntingtin interaction and aggregation at interfaces
    Sharon E. Groover, Adewale Adegbuyiro, Caleb K. Fan, Breanna L. Hodges, Maryssa A. Beasley, Katelyn Taylor, Alyssa R. Stonebraker, Chathuranga Siriwardhana, Justin A. Legleiter · 2021 · Colloids and Surfaces B Biointerfaces
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  17. Old mice present increased levels of succinate dehydrogenase activity and lower vulnerability to dyskinetic effects of 3-nitropropionic acid
    Tatiana Rosado Rosenstock, Vanessa Costhek Abílio, Roberto Frussa‐Filho, Beatriz Hitomi Kiyomoto, Soraya Soubhi Smaili · 2008 · Pharmacology Biochemistry and Behavior
  18. Phosphomimetic Mutations Impact Huntingtin Aggregation in the Presence of a Variety of Lipid Systems
    Sharon E. Groover, Maryssa A. Beasley, Visvanathan Ramamurthy, Justin A. Legleiter · 2020 · Biochemistry
  19. Mutant huntingtin goes straight to the heart
    Elena F. Cattaneo, Paolo Calabresi · 2002 · Nature Neuroscience
  20. Polyglutamine tract‐binding protein‐1 dysfunction induces cell death of neurons through mitochondrial stress
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  21. High-resolution respirometry of fine-needle muscle biopsies in pre-manifest Huntington’s disease expansion mutation carriers shows normal mitochondrial respiratory function
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  23. Late onset vascular dysfunction in the R6/1 model of Huntington’s disease
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  24. Age-associated chromatin relaxation is enhanced in Huntington’s disease mice
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  26. Antioxidants cannot suppress the lethal phenotype of a Drosophila melanogaster model of Huntington’s disease
    Sepehr Bahadorani, Arthur J. Hilliker · 2008 · Genome
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  28. Interactions of amyloidogenic proteins with mitochondrial protein import machinery in aging-related neurodegenerative diseases
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  29. Poration of mitochondrial membranes by amyloidogenic peptides and other biological toxins
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  30. Mutant huntingtin does not cross the mitochondrial outer membrane
    James Hamilton, Tatiana Brustovetsky, Rajesh Khanna, Nickolay Brustovetsky · 2020 · Human Molecular Genetics
  31. Membrane Interactions Accelerate the Self-Aggregation of Huntingtin Exon 1 Fragments in a Polyglutamine Length-Dependent Manner
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  32. Empowering Melatonin Therapeutics with Drosophila Models
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  33. The three sisters of fate: Genetics, pathophysiology and outcomes of animal models of neurodegenerative diseases
    Michael Klonarakis, Maya De Vos, Emma K. Woo, Liam T. Ralph, Jonathan S. Thacker, Joana Gil‐Mohapel · 2022 · Neuroscience & Biobehavioral Reviews
  34. ATF3 plays a protective role against toxicity by N-terminal fragment of mutant huntingtin in stable PC12 cell line
    Yideng Liang, Haibing Jiang, Tamara Ratovitski, Chunfa Jie, Masayuki Nakamura, Ricky R. Hirschhorn, Xiaofang Wang, Wanli Wei Smith, Tsonwin Hai, Michelle A. Poirier et al. · 2009 · Brain Research
  35. 4‐hydroxy tempo improves mitochondrial and neurobehavioral deficits in experimental model of H untington's disease
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  36. Huntington’s Disease Pathogenesis: Mechanisms and Pathways
    Albert R. La Spada, Patrick Weydt, Victor V. Pineda · 2011 · Europe PMC (PubMed Central)
  37. Identification of Novel Therapeutic Targets for Polyglutamine Diseases That Target Mitochondrial Fragmentation
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  38. Mitochondrial distribution violation and nuclear indentations in neurons differentiated from iPSCs of Huntington's disease patients
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  39. Hprt (CAG)146 mice: Age of onset of behavioral abnormalities, time course of neuronal intranuclear inclusion accumulation, neurotransmitter marker alterations, mitochondrial function markers, and susceptibility to 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine
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  41. Short-chain fatty acids in Huntington's disease: Mechanisms of action and their therapeutic implications
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  43. Mitochondrial Biology and the Identification of Biomarkers of Huntington’s Disease
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  45. Involvement of CRMP2 in Regulation of Mitochondrial Morphology and Motility in Huntington’s Disease
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  46. Mitochondrial Dysfunction in Repeat Expansion Diseases
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  47. Metabolic and electrophysiological changes in the basal ganglia of transgenic Huntington's disease rats
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  48. Potential therapeutic targets in polyglutamine-mediated diseases
    Masahisa Katsuno, Hirohisa Watanabe, Masahiko Yamamoto, Gen Sobue · 2014 · Expert Review of Neurotherapeutics
  49. Investigation of tRNALeu/Lys and ATPase 6 Genes Mutations in Huntington’s Disease
    Sadaf Kasraie, Massoud Houshmand, Mohammad Mehdi Banoei, Solmaz Etemad, Mehdi Shafa Shariat Panahi, Parvin Shariati, Mohammad Ali Bahar, Mostafa Moin · 2008 · Cellular and Molecular Neurobiology
  50. Safety, pharmacokinetics and pharmacodynamics of SBT‐020 in patients with early stage Huntington's disease, a 2‐part study
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  52. Huntington Disease
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    David L Blum, Marie‐Christine Galas, Laëtitia Cuvelier, Serge N. Schiffmann · 2003 · Neuroscience Letters
  54. Genetic criteria for Huntington's disease pathogenesis
    James F. Gusella, Marcy E. MacDonald · 2006 · Brain Research Bulletin
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  56. Calcium dysregulation and compensation in cortical pyramidal neurons of the R6/2 mouse model of Huntington’s disease
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  57. Mitochondrial tRNALeu/Lys and ATPase 6/8 Gene Variations in Spinocerebellar Ataxias
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  58. Age-Dependent Biphasic Changes in Ischemic Sensitivity in the Striatum of Huntington's Disease R6/2 Transgenic Mice
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  60. Mitochondrial Dynamics in Neurodegenerative Diseases
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  62. Skeletal muscle characteristics and mitochondrial function in Huntington's disease patients
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  78. Charge within Nt17 peptides modulates huntingtin aggregation and initial lipid binding events
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  79. A high-throughput screening to identify small molecules that suppress huntingtin promoter activity or activate huntingtin-antisense promoter activity
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