Huntington's disease and mitochondrial alterations: emphasis on experimental models

scientific article published on June 2010

Huntington's disease and mitochondrial alterations: emphasis on experimental models is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1007/S10863-010-9289-4
P698PubMed publication ID20524050

P50authorAbel SantamaríaQ88868534
P2093author name stringPaul Carrillo-Mora
Verónica Pérez-De la Cruz
P2860cites workInteraction of the nuclear matrix protein NAKAP with HypA and huntingtin: implications for nuclear toxicity in Huntington's disease pathogenesisQ24300257
A huntingtin-associated protein enriched in brain with implications for pathologyQ24303499
Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicityQ24337727
Excitotoxic damage, disrupted energy metabolism, and oxidative stress in the rat brain: antioxidant and neuroprotective effects of L-carnitineQ46808934
Pyruvate and lactate protect striatal neurons against N-methyl-D-aspartate-induced neurotoxicityQ48098641
Mechanism of kainate toxicity to cerebellar neurons in vitro is analogous to reperfusion tissue injuryQ48202227
Ultrastructural changes in brain parenchyma during normal aging and in animal models of agingQ48226572
Evidence that quinolinic acid severely impairs energy metabolism through activation of NMDA receptors in striatum from developing rats.Q48306465
In vitro effect of quinolinic acid on energy metabolism in brain of young ratsQ48358256
Cyanide-induced generation of oxidative species: involvement of nitric oxide synthase and cyclooxygenase-2.Q48472258
Brain mitochondrial defects amplify intracellular [Ca2+] rise and neurodegeneration but not Ca2+ entry during NMDA receptor activation.Q48600704
ATP as a marker of excitotoxin-induced nerve cell death in vivoQ70041527
Isolated cerebral and cerebellar mitochondria produce free radicals when exposed to elevated CA2+ and Na+: implications for neurodegenerationQ72069850
Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acidQ72098398
Zinc-induced cortical neuronal death: contribution of energy failure attributable to loss of NAD(+) and inhibition of glycolysisQ73704452
Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarizationQ95810356
The uncoupling agent 2,4-dinitrophenol improves mitochondrial homeostasis following striatal quinolinic acid injectionsQ46765141
The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesisQ24616907
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.Q27860836
Genetic interaction between expanded murine Hdh alleles and p53 reveal deleterious effects of p53 on Huntington's disease pathogenesisQ28507529
Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitroQ28584697
HAP1 facilitates effects of mutant huntingtin on inositol 1,4,5-trisphosphate-induced Ca release in primary culture of striatal medium spiny neuronsQ28592352
Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivatorsQ29617353
Mitochondrial control of cell deathQ29617739
N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial traffickingQ30486372
Effects of overexpression of huntingtin proteins on mitochondrial integrityQ30489322
Mitochondria in neurodegeneration: bioenergetic function in cell life and deathQ33541512
Role of protein phosphatases and mitochondria in the neuroprotective effects of estrogensQ33715371
Replicating Huntington's disease phenotype in experimental animalsQ33749061
Mitochondrial dysfunction in the pathogenesis of necrotic and apoptotic cell deathQ33832682
Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcriptionQ33916580
A polymorphic DNA marker genetically linked to Huntington's diseaseQ34255139
The energetics of Huntington's diseaseQ34308028
Behavioral characterization of quinolinate-induced lesions of the medial striatum: relevance for Huntington's diseaseQ34374332
Does impairment of energy metabolism result in excitotoxic neuronal death in neurodegenerative illnesses?Q35254212
Selective defect of in vivo glycolysis in early Huntington's disease striatumQ35634060
Mitochondrial DNA damage is a hallmark of chemically induced and the R6/2 transgenic model of Huntington's diseaseQ35706627
The epidemiology of Huntington's diseaseQ35882166
Recruitment and the role of nuclear localization in polyglutamine-mediated aggregationQ36255989
Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicityQ36320596
Hypothesis: Huntingtin may function in membrane association and vesicular traffickingQ36704515
3-Nitropropionic acid-exogenous animal neurotoxin and possible human striatal toxin.Q36934346
Mitochondrial structural and functional dynamics in Huntington's diseaseQ37356257
3-Nitropropionic acid as a tool to study the mechanisms involved in Huntington's disease: past, present and future.Q37718532
Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradationQ38720406
Mitochondrial-dependent apoptosis in Huntington's disease human cybrids.Q39751441
Oxidative stress and plasma aminopeptidase activity in Huntington's diseaseQ39907630
Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington diseaseQ40031554
Increased oxidative damage and mitochondrial abnormalities in the peripheral blood of Huntington's disease patientsQ40207281
Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequencesQ40231756
Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutationQ40351019
Huntingtin associates with acidic phospholipids at the plasma membraneQ40387540
The early events of oxygen and glucose deprivation: setting the scene for neuronal death?Q40392008
Ca2+-induced permeability transition in human lymphoblastoid cell mitochondria from normal and Huntington's disease individuals.Q40443282
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c releaseQ40551652
Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease.Q40661429
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines.Q40721588
Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcriptionQ40764541
Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c releaseQ40808988
The possible contribution of microglia and macrophages to delayed neuronal death after ischemiaQ40882185
Regional mitochondrial respiratory activity in Huntington's disease brainQ41360902
Complex II inhibition by 3-NP causes mitochondrial fragmentation and neuronal cell death via an NMDA- and ROS-dependent pathway.Q41969689
Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.Q42438521
Energetic dysfunction in quinolinic acid-lesioned rat striatumQ42445269
Excitotoxic brain damage involves early peroxynitrite formation in a model of Huntington's disease in rats: protective role of iron porphyrinate 5,10,15,20-tetrakis (4-sulfonatophenyl)porphyrinate iron (III).Q42482897
Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissuesQ42503942
Cellular and subcellular localization of Huntingtin [corrected] aggregates in the brain of a rat transgenic for Huntington diseaseQ42508306
Cytoplasmic calcium mediates oxidative damage in an excitotoxic /energetic deficit synergic model in rats.Q42526044
Antioxidant strategy to rescue synaptosomes from oxidative damage and energy failure in neurotoxic models in rats: protective role of S-allylcysteineQ43252987
3-nitropropionic acid-induced mitochondrial permeability transition: comparative study of mitochondria from different tissues and brain regionsQ43268781
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's diseaseQ43669934
Oxidative damage and metabolic dysfunction in experimental Huntington's disease: selective vulnerability of the striatum and hippocampus.Q43731488
Pyramidal cell loss in motor cortices in Huntington's diseaseQ44149293
Cyanide induces different modes of death in cortical and mesencephalon cellsQ44186026
In vitro effects of polyglutamine tracts on Ca2+-dependent depolarization of rat and human mitochondria: relevance to Huntington's disease.Q44298045
Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in miceQ44316963
Influence of cytosolic and mitochondrial Ca2+, ATP, mitochondrial membrane potential, and calpain activity on the mechanism of neuron death induced by 3-nitropropionic acidQ44348986
Neuroprotective effects of pyruvate in the quinolinic acid rat model of Huntington's diseaseQ44614374
Protective effect of melatonin on 3-nitropropionic acid-induced oxidative stress in synaptosomes in an animal model of Huntington's diseaseQ45107385
3-nitropropionic acid-induced hydrogen peroxide, mitochondrial DNA damage, and cell death are attenuated by Bcl-2 overexpression in PC12 cellsQ45264432
Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.Q45291767
Targeting oxidative/nitrergic stress ameliorates motor impairment, and attenuates synaptic mitochondrial dysfunction and lipid peroxidation in two models of Huntington's diseaseQ45292072
Treatment with dehydroepiandrosterone prevents oxidative stress induced by 3-nitropropionic acid in synaptosomesQ45292543
Degeneration of pyramidal projection neurons in Huntington's disease cortexQ45294227
Cortical and subcortical glucose consumption measured by PET in patients with Huntington's disease.Q45294680
Clinical Genetics, II. Huntington's disease: from the gene to pathophysiologyQ45294788
Hyperactivity and hypoactivity in a rat model of Huntington's disease: the systemic 3-nitropropionic acid modelQ45295346
1H NMR spectroscopy studies of Huntington's disease: correlations with CAG repeat numbersQ45296001
Distribution of phosphate-activated glutaminase, succinic dehydrogenase, pyruvate dehydrogenase and gamma-glutamyl transpeptidase in post-mortem brain from Huntington's disease and agonal casesQ45296514
Glyceraldehyde 3-phosphate dehydrogenase abnormality in metabolically stressed Huntington disease fibroblasts.Q45296630
Brain glyceraldehyde-3-phosphate dehydrogenase activity in human trinucleotide repeat disordersQ45296636
Biochemical abnormalities and excitotoxicity in Huntington's disease brain.Q45297145
Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtinQ45297269
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's diseaseQ45297400
HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolismQ45298206
Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteinsQ45298596
3-nitropropionic acid induced in vivo protein oxidation in striatal and cortical synaptosomes: insights into Huntington's diseaseQ45299994
Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neuronsQ45300079
Increased apoptosis, Huntingtin inclusions and altered differentiation in muscle cell cultures from Huntington's disease subjectsQ45301339
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 neurodegenerationQ45302924
Wild-type huntingtin participates in protein trafficking between the Golgi and the extracellular spaceQ45303717
Cerebral metabolism and atrophy in huntington's disease determined by18FDG and computed tomographic scanQ45304491
Huntington's disease and mitochondrial DNA deletions: event or regular mechanism for mutant huntingtin protein and CAG repeats expansion?!Q45306758
Mitochondrial NAD+-linked State 3 respiration and complex-I activity are compromised in the cerebral cortex of 3-nitropropionic acid-induced rat model of Huntington's disease.Q45306770
Early nerve ending rescue from oxidative damage and energy failure by L: -carnitine as post-treatment in two neurotoxic models in rat: recovery of antioxidant and reductive capacities.Q45946973
Possible role of sertraline against 3-nitropropionic acid induced behavioral, oxidative stress and mitochondrial dysfunctions in rat brainQ46235939
Melatonin protects against neuronal damage induced by 3-nitropropionic acid in rat striatumQ46480941
Krebs cycle intermediates modulate thiobarbituric acid reactive species (TBARS) production in rat brain in vitro.Q46492101
Quinolinate-induced rat striatal excitotoxicity impairs endoplasmic reticulum Ca2+-ATPase functionQ46728440
P433issue3
P921main subjectHuntington's diseaseQ190564
P304page(s)207-215
P577publication date2010-06-01
P1433published inJournal of Bioenergetics and BiomembranesQ5044470
P1476titleHuntington's disease and mitochondrial alterations: emphasis on experimental models
P478volume42

Reverse relations

Q37749622Mitochondrial matters in Huntington diseasecites workP2860