scholarly article | Q13442814 |
P2093 | author name string | Baskaran Thyagarajan | |
Jonathan H Fox | |||
Sonal Agrawal | |||
Julia Fox | |||
P2860 | cites work | The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice | Q24656405 |
Iron Homeostasis in Health and Disease | Q26775146 | ||
Huntington's disease: underlying molecular mechanisms and emerging concepts | Q27026255 | ||
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. | Q27860836 | ||
Gender and genetic background effects on brain metal levels in APP transgenic and normal mice: implications for Alzheimer beta-amyloid pathology | Q48599293 | ||
Requirement for superoxide in excitotoxic cell death | Q49133494 | ||
Could Conservative Iron Chelation Lead to Neuroprotection in Amyotrophic Lateral Sclerosis? | Q49490050 | ||
Assay for lipid peroxide content in mitochondria by the thiobarbituric acid reaction | Q69428258 | ||
Measurement of iron and copper in biological systems: bleomycin and copper-phenanthroline assays | Q71630063 | ||
Induction of the mitochondrial permeability transition (MPT) by micromolar iron: liberation of calcium is more important than NAD(P)H oxidation | Q84225449 | ||
The chicken or the egg: mitochondrial dysfunction as a cause or consequence of toxicity in Huntington's disease | Q28072937 | ||
Mitochondrial targeting of XJB-5-131 attenuates or improves pathophysiology in HdhQ150 animals with well-developed disease phenotypes | Q28386769 | ||
Inhibition of mitochondrial protein import by mutant huntingtin | Q28394803 | ||
Oxygen consumption deficit in Huntington disease mouse brain under metabolic stress | Q28397230 | ||
Age-dependent changes in the calcium sensitivity of striatal mitochondria in mouse models of Huntington's Disease | Q28512442 | ||
Mitoferrin is essential for erythroid iron assimilation | Q28587125 | ||
Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators | Q29617353 | ||
Haemochromatosis. | Q30251784 | ||
Mouse models of Huntington's disease and methodological considerations for therapeutic trials | Q30481208 | ||
Iron accumulation in the basal ganglia in Huntington's disease: cross-sectional data from the IMAGE-HD study. | Q30946340 | ||
Expression and localization of mitochondrial ferritin mRNA in Alzheimer's disease cerebral cortex | Q31027425 | ||
Selective determination of mitochondrial chelatable iron in viable cells with a new fluorescent sensor | Q31037490 | ||
A simple, highly sensitive and improved method for the measurement of bleomycin-detectable iron: the 'catalytic iron index' and its value in the assessment of iron status in haemochromatosis | Q32061657 | ||
Regional and cellular gene expression changes in human Huntington's disease brain | Q33233457 | ||
Alterations in the levels of iron, ferritin and other trace metals in Parkinson's disease and other neurodegenerative diseases affecting the basal ganglia | Q33322579 | ||
Brain iron chelation by deferiprone in a phase 2 randomised double-blinded placebo controlled clinical trial in Parkinson's disease. | Q33684033 | ||
The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease | Q34011801 | ||
Iron deficiency and iron excess damage mitochondria and mitochondrial DNA in rats. | Q34013010 | ||
Ferroptosis: Death by Lipid Peroxidation | Q34045575 | ||
Ferrostatins inhibit oxidative lipid damage and cell death in diverse disease models | Q34408158 | ||
Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity | Q34634421 | ||
Common dysregulation network in the human prefrontal cortex underlies two neurodegenerative diseases | Q34986607 | ||
Iron accumulates in Huntington's disease neurons: protection by deferoxamine | Q35023030 | ||
Neonatal iron supplementation potentiates oxidative stress, energetic dysfunction and neurodegeneration in the R6/2 mouse model of Huntington's disease. | Q35143644 | ||
Modulation of lipid peroxidation and mitochondrial function improves neuropathology in Huntington's disease mice | Q35152150 | ||
Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease | Q35747131 | ||
Disruption of ATP-binding cassette B8 in mice leads to cardiomyopathy through a decrease in mitochondrial iron export | Q35837234 | ||
Alterations in brain transition metals in Huntington disease: an evolving and intricate story | Q36838442 | ||
Quantitative Susceptibility Mapping Suggests Altered Brain Iron in Premanifest Huntington Disease. | Q36902073 | ||
Neonatal Iron Supplementation Induces Striatal Atrophy in Female YAC128 Huntington's Disease Mice | Q36986770 | ||
VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease | Q37223339 | ||
Friedreich ataxia: neuropathology revised | Q37278583 | ||
Frequency of nuclear mutant huntingtin inclusion formation in neurons and glia is cell-type-specific. | Q37456579 | ||
Oxidative metabolism and Ca2+ handling in isolated brain mitochondria and striatal neurons from R6/2 mice, a model of Huntington's disease | Q37530288 | ||
Iron-dependent functions of mitochondria--relation to neurodegeneration | Q37821334 | ||
Review: iron metabolism and the role of iron in neurodegenerative disorders | Q38155941 | ||
Cysteine-mediated redox signalling in the mitochondria. | Q38292320 | ||
Reduced Mitochondrial Function in Human Huntington Disease Lymphoblasts is Not Due to Alterations in Cardiolipin Metabolism or Mitochondrial Supercomplex Assembly | Q38410393 | ||
Neurodegenerations with Brain Iron Accumulation | Q38576689 | ||
Impact of high iron intake on cognition and neurodegeneration in humans and in animal models: a systematic review | Q38785723 | ||
Frataxin and the molecular mechanism of mitochondrial iron-loading in Friedreich's ataxia | Q38821023 | ||
Management of iron overload in hemoglobinopathies. | Q39412094 | ||
Bioenergetic dysfunction in Huntington's disease human cybrids. | Q39522256 | ||
Alterations in the mitochondrial proteome of neuroblastoma cells in response to complex 1 inhibition. | Q39591876 | ||
Neuroprotective mechanism of mitochondrial ferritin on 6-hydroxydopamine-induced dopaminergic cell damage: implication for neuroprotection in Parkinson's disease. | Q39745493 | ||
Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation | Q39939146 | ||
Contribution of aldehyde dehydrogenase to mitochondrial bioactivation of nitroglycerin: evidence for the activation of purified soluble guanylate cyclase through direct formation of nitric oxide. | Q40514133 | ||
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release | Q40551652 | ||
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. | Q40721588 | ||
Human frataxin maintains mitochondrial iron homeostasis in Saccharomyces cerevisiae. | Q40849308 | ||
Regulating the fate of mRNA: the control of cellular iron metabolism | Q40851091 | ||
Elimination of huntingtin in the adult mouse leads to progressive behavioral deficits, bilateral thalamic calcification, and altered brain iron homeostasis. | Q41187089 | ||
Regional mitochondrial respiratory activity in Huntington's disease brain | Q41360902 | ||
Aceruloplasminemia, an iron metabolic disorder | Q42455448 | ||
Cyclosporine A and MnTMPyP Alleviate α-Synuclein Expression and Aggregation in Cypermethrin-Induced Parkinsonism | Q42703703 | ||
Ring sideroblasts and sideroblastic anemias | Q42743016 | ||
Effects of increased iron intake during the neonatal period on the brain of adult AbetaPP/PS1 transgenic mice. | Q43161323 | ||
Mitochondrial iron and energetic dysfunction distinguish fibroblasts and induced neurons from pantothenate kinase-associated neurodegeneration patients | Q43178831 | ||
Mitochondrial ferritin attenuates β-amyloid-induced neurotoxicity: reduction in oxidative damage through the Erk/P38 mitogen-activated protein kinase pathways | Q43601835 | ||
The iron chelator pyridoxal isonicotinoyl hydrazone inhibits mitochondrial lipid peroxidation induced by Fe(II)-citrate | Q43846361 | ||
Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses | Q44092340 | ||
The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins | Q44092352 | ||
Cypermethrin-induced nigrostriatal dopaminergic neurodegeneration alters the mitochondrial function: a proteomics study | Q44650375 | ||
Iron-sulfur enzyme mediated mitochondrial superoxide toxicity in experimental Parkinson's disease | Q45018861 | ||
Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation | Q45077697 | ||
Abnormal response to cortical activation in early stages of Huntington disease | Q45288459 | ||
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease | Q45289366 | ||
Mitochondrial defect in Huntington's disease caudate nucleus | Q45291734 | ||
Biochemical abnormalities and excitotoxicity in Huntington's disease brain. | Q45297145 | ||
Neuropathological classification of Huntington's disease | Q45297167 | ||
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease | Q45297400 | ||
Decreased expression of striatal signaling genes in a mouse model of Huntington's disease | Q45300376 | ||
Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. | Q45302702 | ||
Ferritin accumulation in dystrophic microglia is an early event in the development of Huntington's disease | Q45305307 | ||
Huntingtin-deficient zebrafish exhibit defects in iron utilization and development. | Q45305391 | ||
Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice. | Q46306829 | ||
Mitoferrin modulates iron toxicity in a Drosophila model of Friedreich's ataxia. | Q47072104 | ||
Towards an Understanding of Energy Impairment in Huntington's Disease Brain | Q47442924 | ||
Clinical Experience With Deferiprone Treatment for Friedreich Ataxia | Q47786184 | ||
Alpha-synuclein induced membrane depolarization and loss of phosphorylation capacity of isolated rat brain mitochondria: implications in Parkinson's disease | Q48274235 | ||
Huntington disease | Q48465200 | ||
Increased murine neonatal iron intake results in Parkinson-like neurodegeneration with age. | Q48502457 | ||
P921 | main subject | Huntington's disease | Q190564 |
P304 | page(s) | 317-329 | |
P577 | publication date | 2018-04-04 | |
P1433 | published in | Free Radical Biology and Medicine | Q5500023 |
P1476 | title | Brain mitochondrial iron accumulates in Huntington's disease, mediates mitochondrial dysfunction, and can be removed pharmacologically | |
P478 | volume | 120 |