| scholarly article | Q13442814 |
| P50 | author | Marcy E. MacDonald | Q67217943 |
| P2093 | author name string | Mathieu Lesort | |
| Peter J Detloff | |||
| Gail V W Johnson | |||
| Yeun Su Choo | |||
| P433 | issue | 14 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | mitochondrion | Q39572 |
| P304 | page(s) | 1407-1420 | |
| P577 | publication date | 2004-05-26 | |
| P1433 | published in | Human Molecular Genetics | Q2720965 |
| P1476 | title | Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release | |
| P478 | volume | 13 |
| Q28742388 | 14-3-3theta protects against neurotoxicity in a cellular Parkinson's disease model through inhibition of the apoptotic factor Bax |
| Q57072860 | A Mitochondria-Associated Oxidative Stress Perspective on Huntington's Disease |
| Q38974551 | A link between chromatin condensation mechanisms and Huntington's disease: connecting the dots. |
| Q34658144 | A novel transferrin/TfR2-mediated mitochondrial iron transport system is disrupted in Parkinson's disease |
| Q33305438 | A role for voltage-dependent anion channel Vdac1 in polyglutamine-mediated neuronal cell death |
| Q45290293 | A study of molecular changes relating to energy metabolism and cellular stress in people with Huntington's disease: looking for biomarkers |
| Q36375845 | Abnormal interaction of VDAC1 with amyloid beta and phosphorylated tau causes mitochondrial dysfunction in Alzheimer's disease |
| Q34627072 | Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage |
| Q92876042 | Alterations of Calcium Channels in a Mouse Model of Huntington's Disease and Neuroprotection by Blockage of CaV1 Channels |
| Q34409630 | Altered Ca(2+) signaling in skeletal muscle fibers of the R6/2 mouse, a model of Huntington's disease |
| Q36913723 | Alternative mitochondrial electron transfer for the treatment of neurodegenerative diseases and cancers: Methylene blue connects the dots. |
| Q33720370 | Angiotensins and Huntington's Disease: A Study on Immortalized Progenitor Striatal Cell Lines |
| Q46919908 | Atorvastatin attenuates mitochondrial toxin-induced striatal degeneration, with decreasing iNOS/c-Jun levels and activating ERK/Akt pathways |
| Q42380694 | BNIP3 Protein Suppresses PINK1 Kinase Proteolytic Cleavage to Promote Mitophagy |
| Q58584663 | Bioenergetics in fibroblasts of patients with Huntington disease are associated with age at onset |
| Q88292931 | Brain mitochondrial iron accumulates in Huntington's disease, mediates mitochondrial dysfunction, and can be removed pharmacologically |
| Q35893954 | Ca(2+) handling in isolated brain mitochondria and cultured neurons derived from the YAC128 mouse model of Huntington's disease |
| Q40020558 | Calcineurin is involved in the early activation of NMDA-mediated cell death in mutant huntingtin knock-in striatal cells |
| Q35906784 | Calcium Handling by Endoplasmic Reticulum and Mitochondria in a Cell Model of Huntington's Disease. |
| Q40031554 | Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington disease |
| Q42199396 | Calcium signaling in neurodegeneration |
| Q35745626 | Cardiac dysfunction in the R6/2 mouse model of Huntington's disease |
| Q50088379 | Cause or compensation?-Altered neuronal Ca2+ handling in Huntington's disease. |
| Q36959352 | Chemotherapy for the brain: the antitumor antibiotic mithramycin prolongs survival in a mouse model of Huntington's disease |
| Q24535603 | Clioquinol down-regulates mutant huntingtin expression in vitro and mitigates pathology in a Huntington's disease mouse model |
| Q41969689 | Complex II inhibition by 3-NP causes mitochondrial fragmentation and neuronal cell death via an NMDA- and ROS-dependent pathway. |
| Q39450953 | Compromised mitochondrial complex II in models of Machado-Joseph disease. |
| Q37619548 | Consensus paper: pathological mechanisms underlying neurodegeneration in spinocerebellar ataxias |
| Q64916070 | Current progress of mitochondrial transplantation that promotes neuronal regeneration. |
| Q40290417 | Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells |
| Q39273647 | Development or disease: duality of the mitochondrial permeability transition pore |
| Q33850154 | Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease. |
| Q36080317 | Dithiol-based compounds maintain expression of antioxidant protein peroxiredoxin 1 that counteracts toxicity of mutant huntingtin |
| Q35839434 | Dopaminergic signaling and striatal neurodegeneration in Huntington's disease |
| Q34659907 | Dysfunctional mitochondrial respiration in the striatum of the Huntington's disease transgenic R6/2 mouse model |
| Q36579470 | Dysregulation of mitochondrial calcium signaling and superoxide flashes cause mitochondrial genomic DNA damage in Huntington disease |
| Q26795417 | Electron Transport Disturbances and Neurodegeneration: From Albert Szent-Györgyi's Concept (Szeged) till Novel Approaches to Boost Mitochondrial Bioenergetics |
| Q30441792 | Elucidating a normal function of huntingtin by functional and microarray analysis of huntingtin-null mouse embryonic fibroblasts |
| Q37834195 | Energy deficit in Huntington disease: why it matters |
| Q48195020 | Expanded and Wild-type Ataxin-3 Modify the Redox Status of SH-SY5Y Cells Overexpressing α-Synuclein |
| Q36861129 | Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease. |
| Q37473242 | Ginsenosides protect striatal neurons in a cellular model of Huntington's disease |
| Q37411283 | Glutathione peroxidase 4 differentially regulates the release of apoptogenic proteins from mitochondria |
| Q37735155 | Glyceraldehyde-3-phosphate Dehydrogenase (GAPDH) Aggregation Causes Mitochondrial Dysfunction during Oxidative Stress-induced Cell Death. |
| Q28573381 | Heterogeneous nuclear ribonucleoprotein K enhances insulin-induced expression of mitochondrial UCP2 protein |
| Q33562672 | High-resolution respirometry of fine-needle muscle biopsies in pre-manifest Huntington's disease expansion mutation carriers shows normal mitochondrial respiratory function. |
| Q40717667 | Huntingtin is required for ciliogenesis and neurogenesis during early Xenopus development |
| Q33945927 | Huntingtin protein is essential for mitochondrial metabolism, bioenergetics and structure in murine embryonic stem cells |
| Q98471370 | Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities |
| Q39390180 | Huntington's Disease and Mitochondria |
| Q39456024 | Huntington's Disease: Calcium Dyshomeostasis and Pathology Models |
| Q39035495 | Huntington's Disease: Mechanisms of Pathogenesis and Therapeutic Strategies |
| Q37820343 | Huntington's disease and Group I metabotropic glutamate receptors |
| Q34319766 | Huntington's disease and its therapeutic target genes: a global functional profile based on the HD Research Crossroads database |
| Q42472433 | Huntington's disease and mitochondrial alterations: emphasis on experimental models |
| Q37889820 | Huntington's disease, calcium, and mitochondria |
| Q37016096 | Huntington's disease: progress and potential in the field |
| Q36159463 | Impaired GAPDH-induced mitophagy contributes to the pathology of Huntington's disease |
| Q37323316 | Impaired PGC-1alpha function in muscle in Huntington's disease. |
| Q64075331 | Impaired Redox Signaling in Huntington's Disease: Therapeutic Implications |
| Q37466100 | Impaired mitochondrial trafficking in Huntington's disease |
| Q37142702 | Impaired regulation of brain mitochondria by extramitochondrial Ca2+ in transgenic Huntington disease rats |
| Q34042260 | Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation |
| Q39460807 | In vitro and in vivo aggregation of a fragment of huntingtin protein directly causes free radical production |
| Q36276521 | Increased TRPC5 glutathionylation contributes to striatal neuron loss in Huntington's disease |
| Q34313991 | Increased mitochondrial fission and neuronal dysfunction in Huntington's disease: implications for molecular inhibitors of excessive mitochondrial fission |
| Q28394803 | Inhibition of mitochondrial protein import by mutant huntingtin |
| Q34483577 | Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin |
| Q37575814 | Involvement of the mitochondrial permeability transition pore in chronic ethanol-mediated liver injury in mice |
| Q26829932 | Iron dysregulation in Huntington's disease |
| Q42616331 | Keratins modulate the shape and function of hepatocyte mitochondria: a mechanism for protection from apoptosis |
| Q46832266 | L-type calcium channel blocker ameliorates diabetic encephalopathy by modulating dysregulated calcium homeostasis |
| Q90684471 | Lipid Membranes Influence the Ability of Small Molecules To Inhibit Huntingtin Fibrillization |
| Q39014909 | Localized changes to glycogen synthase kinase-3 and collapsin response mediator protein-2 in the Huntington's disease affected brain. |
| Q64969037 | Mechanisms of protein toxicity in neurodegenerative diseases. |
| Q34140579 | Metabolic state determines sensitivity to cellular stress in Huntington disease: normalization by activation of PPARγ. |
| Q28394301 | Metabolism in HD: still a relevant mechanism? |
| Q38239515 | Metabotropic glutamate receptor 5 as a potential therapeutic target in Huntington's disease |
| Q42465415 | Metabotropic glutamate receptor-mediated cell signaling pathways are altered in a mouse model of Huntington's disease |
| Q37535535 | Mitochondria and energetic depression in cell pathophysiology |
| Q36745271 | Mitochondria in neurodegeneration |
| Q37216404 | Mitochondria in neuroplasticity and neurological disorders |
| Q24646020 | Mitochondria, calcium and cell death: a deadly triad in neurodegeneration |
| Q26770200 | Mitochondria-associated membranes as hubs for neurodegeneration |
| Q36095330 | Mitochondrial Ca(2+) and neurodegeneration |
| Q64074984 | Mitochondrial Dysfunction in Huntington's Disease; Interplay Between HSF1, p53 and PGC-1α Transcription Factors |
| Q55081064 | Mitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's Disease. |
| Q64986417 | Mitochondrial Respiratory Function in Peripheral Blood Cells from Huntington's Disease Patients. |
| Q28580981 | Mitochondrial aconitase is a transglutaminase 2 substrate: transglutamination is a probable mechanism contributing to high-molecular-weight aggregates of aconitase and loss of aconitase activity in Huntington disease brain |
| Q37965763 | Mitochondrial and metabolic-based protective strategies in Huntington's disease: the case of creatine and coenzyme Q. |
| Q37348590 | Mitochondrial approaches for neuroprotection |
| Q37747170 | Mitochondrial bioenergetics and dynamics in Huntington's disease: tripartite synapses and selective striatal degeneration |
| Q97884849 | Mitochondrial calcium signalling and neurodegenerative diseases |
| Q34046887 | Mitochondrial calcium uptake capacity as a therapeutic target in the R6/2 mouse model of Huntington's disease |
| Q61453825 | Mitochondrial distribution violation and nuclear indentations in neurons differentiated from iPSCs of Huntington's disease patients |
| Q41998875 | Mitochondrial division inhibitor 1 protects against mutant huntingtin-induced abnormal mitochondrial dynamics and neuronal damage in Huntington's disease |
| Q38237889 | Mitochondrial dynamic changes in health and genetic diseases |
| Q34442070 | Mitochondrial dysfunction and apoptosis underlie the pathogenic process in alpha-B-crystallin desmin-related cardiomyopathy |
| Q28269333 | Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases |
| Q92479828 | Mitochondrial dysfunction in neurodegenerative diseases and the potential countermeasure |
| Q38086474 | Mitochondrial dysfunction in psychiatric and neurological diseases: cause(s), consequence(s), and implications of antioxidant therapy |
| Q34605764 | Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli |
| Q37750542 | Mitochondrial functional alterations in relation to pathophysiology of Huntington's disease |
| Q28397548 | Mitochondrial lipids in neurodegeneration |
| Q33580327 | Mitochondrial matters of the brain: the role in Huntington's disease |
| Q39014966 | Mitochondrial membrane fluidity is consistently increased in different models of Huntington disease: restorative effects of olesoxime |
| Q37427280 | Mitochondrial permeability transition pore induces mitochondria injury in Huntington disease. |
| Q37732485 | Mitochondrial quality control and neurological disease: an emerging connection |
| Q45307199 | Mitochondrial sensitivity and altered calcium handling underlie enhanced NMDA-induced apoptosis in YAC128 model of Huntington's disease. |
| Q37356257 | Mitochondrial structural and functional dynamics in Huntington's disease |
| Q45302980 | Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: effect of histone deacetylase inhibitors. |
| Q37776087 | Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease |
| Q27005950 | Mouse models of polyglutamine diseases in therapeutic approaches: review and data table. Part II. |
| Q38458943 | Mutant Huntingtin and Elusive Defects in Oxidative Metabolism and Mitochondrial Calcium Handling |
| Q45294777 | Mutant huntingtin and glycogen synthase kinase 3-beta accumulate in neuronal lipid rafts of a presymptomatic knock-in mouse model of Huntington's disease |
| Q37308357 | Mutant huntingtin and mitochondrial dysfunction |
| Q34634421 | Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity |
| Q92185503 | Mutant huntingtin disrupts mitochondrial proteostasis by interacting with TIM23 |
| Q40231756 | Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences |
| Q35747131 | Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease |
| Q35645271 | Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease |
| Q35846393 | Mutations in cytochrome c oxidase subunit VIa cause neurodegeneration and motor dysfunction in Drosophila |
| Q45306350 | N-Acetylcysteine reverses mitochondrial dysfunctions and behavioral abnormalities in 3-nitropropionic acid-induced Huntington's disease |
| Q30486372 | N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking |
| Q38074521 | Neuronal Ca(2+) dyshomeostasis in Huntington disease. |
| Q38179996 | Neuronal calcium signaling: function and dysfunction |
| Q35072356 | Neuronal store-operated calcium entry pathway as a novel therapeutic target for Huntington's disease treatment |
| Q26775096 | Old Things New View: Ascorbic Acid Protects the Brain in Neurodegenerative Disorders |
| Q45290815 | Olesoxime suppresses calpain activation and mutant huntingtin fragmentation in the BACHD rat. |
| Q39035184 | Oxidative metabolism and Ca2+ handling in striatal mitochondria from YAC128 mice, a model of Huntington's disease |
| Q30583566 | PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function. |
| Q27001671 | PGC-1α, mitochondrial dysfunction, and Huntington's disease |
| Q24312713 | Parkin, PINK1, and DJ-1 form a ubiquitin E3 ligase complex promoting unfolded protein degradation |
| Q28267631 | Polyglutamine tract-binding protein-1 dysfunction induces cell death of neurons through mitochondrial stress |
| Q33580733 | Potent inhibition of huntingtin aggregation and cytotoxicity by a disulfide bond-free single-domain intracellular antibody |
| Q45298703 | Progressive hepatic mitochondrial dysfunction in premanifest Huntington's disease |
| Q36253348 | Promethazine protects against 3-nitropropionic acid-induced neurotoxicity. |
| Q37739093 | Protein Quality Control by Molecular Chaperones in Neurodegeneration |
| Q37775036 | Proteomic analysis of mitochondrial dysfunction in neurodegenerative diseases |
| Q30418699 | Proteomic analysis of wild-type and mutant huntingtin-associated proteins in mouse brains identifies unique interactions and involvement in protein synthesis |
| Q45305834 | Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease. |
| Q24317318 | Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity |
| Q26829211 | Role of cerebral cortex in the neuropathology of Huntington's disease |
| Q37557789 | Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease |
| Q28386202 | Role of oxidative DNA damage in mitochondrial dysfunction and Huntington's disease pathogenesis |
| Q37951338 | Role of p53 in neurodegenerative diseases |
| Q41714466 | Roles of sigma-1 receptors on mitochondrial functions relevant to neurodegenerative diseases |
| Q38835914 | Rosiglitazone activation of PPARγ-dependent signaling is neuroprotective in mutant huntingtin expressing cells |
| Q37204122 | S-nitrosylation of dynamin-related protein 1 mediates mutant huntingtin-induced mitochondrial fragmentation and neuronal injury in Huntington's disease |
| Q47710910 | Searching for Correlations Between the Development of Neurodegenerative Hallmarks: Targeting Huntingtin as a Contributing Factor |
| Q36597920 | Selective neuronal degeneration in Huntington's disease |
| Q28384036 | Shaping the role of mitochondria in the pathogenesis of Huntington's disease |
| Q96230762 | Shedding a new light on Huntington's disease: how blood can both propagate and ameliorate disease pathology |
| Q36483870 | Signaling pathways regulating neuron-glia interaction and their implications in Alzheimer's disease |
| Q37839534 | Small changes, big impact: posttranslational modifications and function of huntingtin in Huntington disease. |
| Q37140780 | Spinocerebellar ataxias caused by polyglutamine expansions: a review of therapeutic strategies |
| Q27679485 | Structure and Topology of the Huntingtin 1–17 Membrane Anchor by a Combined Solution and Solid-State NMR Approach |
| Q38035753 | Taurine and central nervous system disorders |
| Q91644271 | The Interplay between Ca2+ Signaling Pathways and Neurodegeneration |
| Q57896358 | The Role of Adenosine Tone and Adenosine Receptors in Huntington's Disease |
| Q64069200 | The Role of the Antioxidant Response in Mitochondrial Dysfunction in Degenerative Diseases: Cross-Talk between Antioxidant Defense, Autophagy, and Apoptosis |
| Q38887200 | The antioxidant protein Oxr1 influences aspects of mitochondrial morphology |
| Q45290121 | The biology of Huntington's disease. |
| Q28072937 | The chicken or the egg: mitochondrial dysfunction as a cause or consequence of toxicity in Huntington's disease |
| Q37798079 | The clinical and genetic features of Huntington disease |
| Q44376761 | The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis. |
| Q37765841 | The interrelationship between mitochondrial dysfunction and transcriptional dysregulation in Huntington disease |
| Q37223251 | The methyl donor S-adenosylmethionine prevents liver hypoxia and dysregulation of mitochondrial bioenergetic function in a rat model of alcohol-induced fatty liver disease |
| Q36775895 | The mitochondrial permeability transition in neurologic disease |
| Q37781736 | The role of deubiquitinating enzymes in apoptosis |
| Q36521932 | The role of mitochondria in inherited neurodegenerative diseases |
| Q36135714 | The therapeutic potential of mitochondrial channels in cancer, ischemia-reperfusion injury, and neurodegeneration |
| Q36358871 | The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies |
| Q27022757 | Therapeutic Approaches for Inhibition of Protein Aggregation in Huntington's Disease |
| Q34411338 | Therapeutic approaches to preventing cell death in Huntington disease |
| Q24618141 | Tissue transglutaminase overexpression does not modify the disease phenotype of the R6/2 mouse model of Huntington's disease |
| Q36379946 | Transducer of regulated CREB-binding proteins (TORCs) transcription and function is impaired in Huntington's disease |
| Q38234883 | Ubiquitin pathways in neurodegenerative disease |
| Q28469280 | Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolism |
| Q45299293 | VCP cooperates with UBXD1 to degrade mitochondrial outer membrane protein MCL1 in model of Huntington's disease |
Search more.