| scholarly article | Q13442814 |
| P50 | author | Xingshun Xu | Q39706435 |
| P2093 | author name string | He Li | |
| Jianjun Wang | |||
| Xiao-Jiang Li | |||
| Shihua Li | |||
| Adam L Orr | |||
| Chuan-En Wang | |||
| J Timothy Greenamyre | |||
| Juan Rong | |||
| Pier Giorgio Mastroberardino | |||
| P2860 | cites work | Mitochondrial dysfunction in Huntington's disease: the bioenergetics of isolated and in situ mitochondria from transgenic mice | Q45304684 |
| Polyglutamine expansion inhibits respiration by increasing reactive oxygen species in isolated mitochondria | Q46904704 | ||
| A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. | Q27860836 | ||
| Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases | Q28269333 | ||
| Neurological abnormalities in a knock-in mouse model of Huntington's disease | Q28508179 | ||
| Age-dependent changes in the calcium sensitivity of striatal mitochondria in mouse models of Huntington's Disease | Q28512442 | ||
| Expression of huntingtin-associated protein-1 in neuronal cells implicates a role in neuritic growth | Q28565508 | ||
| Interaction of Huntingtin-associated protein-1 with kinesin light chain: implications in intracellular trafficking in neurons | Q28573203 | ||
| Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro | Q28584697 | ||
| Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules | Q28590634 | ||
| Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain | Q29617982 | ||
| Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis. | Q33948101 | ||
| The presynaptic particle web: ultrastructure, composition, dissolution, and reconstitution | Q34095414 | ||
| Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin | Q34483577 | ||
| Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity | Q36320596 | ||
| Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity. | Q36324397 | ||
| Multiple pathways contribute to the pathogenesis of Huntington disease | Q36686156 | ||
| Oxidative stress and mitochondrial dysfunction in neurodegenerative diseases | Q36736582 | ||
| Huntington's disease. Pathogenesis and management | Q39460676 | ||
| The cellular and subcellular localization of huntingtin-associated protein 1 (HAP1): comparison with huntingtin in rat and human. | Q39758563 | ||
| Context-dependent dysregulation of transcription by mutant huntingtin | Q40225463 | ||
| Mitochondrial localization of mu-calpain | Q40356308 | ||
| Mitochondrial localization of cyclooxygenase-2 and calcium-independent phospholipase A2 in human cancer cells: implication in apoptosis resistance | Q40426140 | ||
| Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release | Q40551652 | ||
| Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. | Q40661429 | ||
| Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. | Q40704833 | ||
| Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions | Q40709427 | ||
| Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. | Q40721588 | ||
| Calpain activation in Huntington's disease. | Q40723358 | ||
| Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats | Q41041541 | ||
| Cellular and subcellular localization of Huntingtin [corrected] aggregates in the brain of a rat transgenic for Huntington disease | Q42508306 | ||
| Rapid isolation of metabolically active mitochondria from rat brain and subregions using Percoll density gradient centrifugation | Q43950113 | ||
| The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis. | Q44376761 | ||
| Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release | Q44545687 | ||
| Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in Drosophila | Q44607268 | ||
| Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport | Q44607270 | ||
| Neuropathological classification of Huntington's disease | Q45297167 | ||
| Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin | Q45297269 | ||
| Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. | Q45297497 | ||
| Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons | Q45300079 | ||
| Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin | Q45301569 | ||
| Huntingtin's critical cleavage | Q45302284 | ||
| Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. | Q45302702 | ||
| Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration | Q45302924 | ||
| Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: effect of histone deacetylase inhibitors. | Q45302980 | ||
| P4510 | describes a project that uses | ImageJ | Q1659584 |
| P433 | issue | 11 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | mitochondrion | Q39572 |
| P304 | page(s) | 2783-2792 | |
| P577 | publication date | 2008-03-01 | |
| P1433 | published in | Journal of Neuroscience | Q1709864 |
| P1476 | title | N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking | |
| P478 | volume | 28 |
| Q38974551 | A link between chromatin condensation mechanisms and Huntington's disease: connecting the dots. |
| Q38174451 | A mitochondrial basis for Huntington's disease: therapeutic prospects |
| Q36466769 | A refined analysis of superoxide production by mitochondrial sn-glycerol 3-phosphate dehydrogenase. |
| Q37153804 | A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin |
| Q37607449 | Abnormal mitochondrial dynamics and neurodegenerative diseases |
| Q34627072 | Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage |
| Q37681370 | Abnormal mitochondrial dynamics--a novel therapeutic target for Alzheimer's disease? |
| Q37323312 | Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease. |
| Q35912172 | Altered lysosomal positioning affects lysosomal functions in a cellular 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 |
| Q34181169 | Assessing the contribution of heterogeneous distributions of oligomers to aggregation mechanisms of polyglutamine peptides |
| Q34287719 | Autophagy of mitochondria: a promising therapeutic target for neurodegenerative disease |
| Q41494079 | Bioenergetic analysis of isolated cerebrocortical nerve terminals on a microgram scale: spare respiratory capacity and stochastic mitochondrial failure |
| Q35893954 | Ca(2+) handling in isolated brain mitochondria and cultured neurons derived from the YAC128 mouse model of Huntington's disease |
| Q35906784 | Calcium Handling by Endoplasmic Reticulum and Mitochondria in a Cell Model of Huntington's Disease. |
| Q26866552 | Calcium channel blockers and Alzheimer's disease |
| Q90140618 | Cell-Autonomous and Non-cell-Autonomous Pathogenic Mechanisms in Huntington's Disease: Insights from In Vitro and In Vivo Models |
| Q30787791 | Cholesterol Modifies Huntingtin Binding to, Disruption of, and Aggregation on Lipid Membranes |
| Q37476957 | Circadian dysfunction in response to in vivo treatment with the mitochondrial toxin 3-nitropropionic acid |
| Q30496117 | Coenzyme Q protects Caenorhabditis elegans GABA neurons from calcium-dependent degeneration |
| Q33619310 | Complexity and heterogeneity: what drives the ever-changing brain in Huntington's disease? |
| Q37760791 | Current understanding on the pathogenesis of polyglutamine diseases |
| Q30425063 | Deletion of the huntingtin proline-rich region does not significantly affect normal huntingtin function in mice |
| Q33931283 | Differential vulnerability of neurons in Huntington's disease: the role of cell type-specific features |
| Q39809646 | Disruption of Rab11 activity in a knock-in mouse model of Huntington's disease |
| Q38116463 | Do we age because we have mitochondria? |
| Q55295842 | Dual Therapy with Liraglutide and Ghrelin Promotes Brain and Peripheral Energy Metabolism in the R6/2 Mouse Model of Huntington's Disease. |
| Q34659907 | Dysfunctional mitochondrial respiration in the striatum of the Huntington's disease transgenic R6/2 mouse model |
| Q30489322 | Effects of overexpression of huntingtin proteins on mitochondrial integrity |
| Q37580731 | Endogenous neuroprotection in chronic neurodegenerative disorders: with particular regard to the kynurenines |
| Q37834195 | Energy deficit in Huntington disease: why it matters |
| Q38013005 | Energy dysfunction in Huntington's disease: insights from PGC-1α, AMPK, and CKB. |
| Q45942404 | Enhanced sensitivity of striatal neurons to axonal transport defects induced by mutant huntingtin. |
| Q44933645 | Essential sequence of the N-terminal cytoplasmic localization-related domain of huntingtin and its effect on huntingtin aggregates |
| Q64227126 | Exploring the molecular interface between hypoxia-inducible factor signalling and mitochondria |
| Q38259591 | From pathways to targets: understanding the mechanisms behind polyglutamine disease. |
| Q28468383 | HDAC6 Inhibitors Rescued the Defective Axonal Mitochondrial Movement in Motor Neurons Derived from the Induced Pluripotent Stem Cells of Peripheral Neuropathy Patients with HSPB1 Mutation |
| Q28390800 | Homeostatic adaptations in brain energy metabolism in mouse models of Huntington disease |
| Q42592492 | Hsp60 chaperonin acts as barrier to pharmacologically induced oxidative stress mediated apoptosis in tumor cells with differential stress response |
| Q27332460 | Human A53T α-synuclein causes reversible deficits in mitochondrial function and dynamics in primary mouse cortical neurons |
| Q33529116 | Huntingtin interacts with the cue domain of gp78 and inhibits gp78 binding to ubiquitin and p97/VCP. |
| Q34274478 | Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis. |
| Q98471370 | Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities |
| Q38267443 | Huntington's Disease: An Immune Perspective. |
| Q39035495 | Huntington's Disease: Mechanisms of Pathogenesis and Therapeutic Strategies |
| 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 |
| Q27026255 | Huntington's disease: underlying molecular mechanisms and emerging concepts |
| Q45296557 | IGF-1 intranasal administration rescues Huntington's disease phenotypes in YAC128 mice. |
| Q36159463 | Impaired GAPDH-induced mitophagy contributes to the pathology of Huntington's disease |
| Q37323316 | Impaired PGC-1alpha function in muscle in Huntington's disease. |
| Q37466100 | Impaired mitochondrial trafficking in Huntington's disease |
| Q34042260 | Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation |
| 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 |
| Q24644954 | Intracellular degradation of misfolded proteins in polyglutamine neurodegenerative diseases |
| Q57454820 | Introducing an expanded CAG tract into the huntingtin gene causes a wide spectrum of ultrastructural defects in cultured human cells |
| Q44742505 | Investigation of membrane penetration depth and interactions of the amino-terminal domain of huntingtin: refined analysis by tryptophan fluorescence measurement |
| 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. |
| Q36149988 | Measures of growth in children at risk for Huntington disease |
| Q64969037 | Mechanisms of protein toxicity in neurodegenerative diseases. |
| Q41949133 | Metabolic Stress and Disorders Related to Alterations in Mitochondrial Fission or Fusion |
| Q28394301 | Metabolism in HD: still a relevant mechanism? |
| Q35807409 | Mitochondria as a therapeutic target for aging and neurodegenerative diseases |
| Q92738481 | Mitochondria in neurodegenerative diseases |
| Q37216404 | Mitochondria in neuroplasticity and neurological disorders |
| Q24646020 | Mitochondria, calcium and cell death: a deadly triad in neurodegeneration |
| Q37466059 | Mitochondria: a therapeutic target in neurodegeneration |
| Q36124905 | Mitochondria: the next (neurode)generation |
| Q37355444 | Mitochondrial DNA damage is associated with reduced mitochondrial bioenergetics in Huntington's disease. |
| Q47888701 | Mitochondrial Etiology of Neuropsychiatric Disorders. |
| 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. |
| Q64966341 | Mitochondrial alterations accompanied by oxidative stress conditions in skin fibroblasts of Huntington's disease patients. |
| Q37348590 | Mitochondrial approaches for neuroprotection |
| Q37747170 | Mitochondrial bioenergetics and dynamics in Huntington's disease: tripartite synapses and selective striatal degeneration |
| Q37418288 | Mitochondrial calcium function and dysfunction in the central nervous system |
| Q34046887 | Mitochondrial calcium uptake capacity as a therapeutic target in the R6/2 mouse model of Huntington's disease |
| Q83827338 | Mitochondrial diseases |
| Q21710687 | Mitochondrial disturbances, excitotoxicity, neuroinflammation and kynurenines: Novel therapeutic strategies for neurodegenerative disorders |
| Q41998875 | Mitochondrial division inhibitor 1 protects against mutant huntingtin-induced abnormal mitochondrial dynamics and neuronal damage in Huntington's disease |
| Q29615646 | Mitochondrial dynamics--fusion, fission, movement, and mitophagy--in neurodegenerative diseases |
| Q92479828 | Mitochondrial dysfunction in neurodegenerative diseases and the potential countermeasure |
| 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 |
| Q37356319 | Mitochondrial kinases in Parkinson's disease: converging insights from neurotoxin and genetic models |
| Q35606693 | Mitochondrial medicine for aging and neurodegenerative diseases |
| Q39014966 | Mitochondrial membrane fluidity is consistently increased in different models of Huntington disease: restorative effects of olesoxime |
| Q37732485 | Mitochondrial quality control and neurological disease: an emerging connection |
| Q37356257 | Mitochondrial structural and functional dynamics in Huntington's disease |
| Q27310252 | Mitochondrial transport in neurons: impact on synaptic homeostasis and neurodegeneration |
| Q92547045 | Mitochondrial transport serves as a mitochondrial quality control strategy in axons: Implications for central nervous system disorders |
| Q92633565 | Mitophagy in Alzheimer's Disease and Other Age-Related Neurodegenerative Diseases |
| Q35152150 | Modulation of lipid peroxidation and mitochondrial function improves neuropathology in Huntington's disease mice |
| Q39407508 | Modulation of polyglutamine conformations and dimer formation by the N-terminus of huntingtin |
| 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. |
| Q24605880 | Multiple Aspects of Gene Dysregulation in Huntington's Disease |
| 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 |
| 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 |
| Q64273045 | Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription |
| 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 |
| Q45306350 | N-Acetylcysteine reverses mitochondrial dysfunctions and behavioral abnormalities in 3-nitropropionic acid-induced Huntington's disease |
| Q99633330 | Neural stem cells derived from the developing forebrain of YAC128 mice exhibit pathological features of Huntington's disease |
| Q34373713 | Nicotinamide improves motor deficits and upregulates PGC-1α and BDNF gene expression in a mouse model of Huntington's disease |
| Q37540341 | Nuclear accumulation of polyglutamine disease proteins and neuropathology |
| Q45290815 | Olesoxime suppresses calpain activation and mutant huntingtin fragmentation in the BACHD rat. |
| Q45289374 | Optical coherence tomography findings in Huntington's disease: a potential biomarker of disease progression |
| Q37530288 | Oxidative metabolism and Ca2+ handling in isolated brain mitochondria and striatal neurons from R6/2 mice, a model of Huntington's disease |
| Q35924062 | Oxidative metabolism in YAC128 mouse model of Huntington's disease |
| Q24635310 | Oxidative stress, mitochondrial dysfunction, and aging |
| Q28397230 | Oxygen consumption deficit in Huntington disease mouse brain under metabolic stress |
| 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 |
| Q26853311 | Parkinson's disease, insulin resistance and novel agents of neuroprotection |
| Q45304677 | Post-translational modifications clustering within proteolytic domains decrease mutant huntingtin toxicity |
| Q37818887 | Proteasomal dysfunction in aging and Huntington disease |
| Q41841553 | Protective Effects of Aqueous Extract of Luehea divaricata against Behavioral and Oxidative Changes Induced by 3-Nitropropionic Acid in Rats. |
| Q39124557 | Proteins Containing Expanded Polyglutamine Tracts and Neurodegenerative Disease |
| Q40355188 | Prothymosin-α interacts with mutant huntingtin and suppresses its cytotoxicity in cell culture. |
| Q41377417 | Quantitative Proteomic Analysis Reveals Similarities between Huntington's Disease (HD) and Huntington's Disease-Like 2 (HDL2) Human Brains |
| Q38855572 | Quercetin improves the activity of the ubiquitin-proteasomal system in 150Q mutated huntingtin-expressing cells but exerts detrimental effects on neuronal survivability |
| Q55414200 | RNA Aptamers Rescue Mitochondrial Dysfunction in a Yeast Model of Huntington’s Disease. |
| Q36518622 | Reducing Lissencephaly-1 levels augments mitochondrial transport and has a protective effect in adult Drosophila neurons. |
| Q57138614 | Removal of the Mitochondrial Fission Factor Mff Exacerbates Neuronal Loss and Neurological Phenotypes in a Huntington's Disease Mouse Model |
| Q28386202 | Role of oxidative DNA damage in mitochondrial dysfunction and Huntington's disease pathogenesis |
| Q28854315 | Rotenone-dependent changes of anterograde motor protein expression and mitochondrial mobility in brain areas related to neurodegenerative diseases |
| Q36279816 | Serum Response Factor (SRF)-cofilin-actin signaling axis modulates mitochondrial dynamics |
| Q28384036 | Shaping the role of mitochondria in the pathogenesis of Huntington's disease |
| Q41457916 | Soluble N-terminal fragment of mutant Huntingtin protein impairs mitochondrial axonal transport in cultured hippocampal neurons |
| Q33831774 | Striatal Vulnerability in Huntington's Disease: Neuroprotection Versus Neurotoxicity |
| Q47606530 | Structure, function, and regulation of mitofusin-2 in health and disease |
| Q26752720 | Subcellular Clearance and Accumulation of Huntington Disease Protein: A Mini-Review |
| Q60922394 | Targeting the proteostasis network in Huntington's disease |
| Q35542857 | The Interface between Cytoskeletal Aberrations and Mitochondrial Dysfunction in Alzheimer's Disease and Related Disorders |
| Q37969749 | The biological function of the Huntingtin protein and its relevance to Huntington's Disease pathology |
| Q38103448 | The causative role and therapeutic potential of the kynurenine pathway in neurodegenerative disease. |
| Q26853454 | The dynamics of the mitochondrial organelle as a potential therapeutic target |
| Q41154714 | The huntingtin N17 domain is a multifunctional CRM1 and Ran-dependent nuclear and cilial export signal |
| Q28591685 | The regulation of autophagosome dynamics by huntingtin and HAP1 is disrupted by expression of mutant huntingtin, leading to defective cargo degradation |
| Q37878430 | The role of mitochondria in neurodegenerative diseases |
| Q38532744 | The role of mitochondrial disturbances in Alzheimer, Parkinson and Huntington diseases |
| Q35219902 | The striatum is highly susceptible to mitochondrial oxidative phosphorylation dysfunctions |
| Q64859904 | Therapeutic approaches to Huntington disease: from the bench to the clinic |
| Q47442924 | Towards an Understanding of Energy Impairment in Huntington's Disease Brain |
| Q36379946 | Transducer of regulated CREB-binding proteins (TORCs) transcription and function is impaired in Huntington's disease |
| Q26742053 | Transporting mitochondria in neurons |
| Q34775357 | Type 2 transglutaminase in Huntington’s disease: a double‐edged sword with clinical potential |
| Q38950512 | UPS Activation in the Battle Against Aging and Aggregation-Related Diseases: An Extended Review |
| Q27692704 | Ubiquitin-dependent mitochondrial protein degradation |
| Q21129327 | Ubiquitin-proteasome system involvement in Huntington's disease |
| Q99727449 | pS421 huntingtin modulates mitochondrial phenotypes and confers neuroprotection in an HD hiPSC model |
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