| scholarly article | Q13442814 |
| P50 | author | Rajnish Kumar Chaturvedi | Q38798873 |
| M. Flint Beal | Q67409129 | ||
| P2093 | author name string | Lichuan Yang | |
| Noel Y Calingasan | |||
| Ashu Johri | |||
| Thomas Hennessey | |||
| P2860 | cites work | The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription | Q22254119 |
| Peroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulator | Q24294798 | ||
| AMP kinase is required for mitochondrial biogenesis in skeletal muscle in response to chronic energy deprivation | Q24541475 | ||
| Resveratrol delays age-related deterioration and mimics transcriptional aspects of dietary restriction without extending life span | Q24649811 | ||
| PGC-1alpha deficiency causes multi-system energy metabolic derangements: muscle dysfunction, abnormal weight control and hepatic steatosis | Q24798075 | ||
| A cold-inducible coactivator of nuclear receptors linked to adaptive thermogenesis | Q27860471 | ||
| Resveratrol improves health and survival of mice on a high-calorie diet | Q27860950 | ||
| Resveratrol improves mitochondrial function and protects against metabolic disease by activating SIRT1 and PGC-1alpha | Q28274682 | ||
| Striatal glucose metabolism and dopamine D2 receptor binding in asymptomatic gene carriers and patients with Huntington's disease | Q28302222 | ||
| PGC-1{alpha} and PGC-1{beta} regulate mitochondrial density in neurons | Q28583359 | ||
| Transcriptional co-activator PGC-1 alpha drives the formation of slow-twitch muscle fibres | Q29555845 | ||
| Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice | Q29614547 | ||
| CREB regulates hepatic gluconeogenesis through the coactivator PGC-1 | Q29615692 | ||
| Metabolic control through the PGC-1 family of transcription coactivators | Q29616509 | ||
| Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators | Q29617353 | ||
| AMP-activated protein kinase (AMPK) action in skeletal muscle via direct phosphorylation of PGC-1alpha | Q29620443 | ||
| N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking | Q30486372 | ||
| Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease | Q33292417 | ||
| Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates | Q34103541 | ||
| Overexpression of peroxisome proliferator-activated receptor gamma co-activator-1alpha leads to muscle atrophy with depletion of ATP | Q34571312 | ||
| Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin | Q35013054 | ||
| Transcriptional abnormalities in Huntington disease | Q35113363 | ||
| Transcriptional regulatory circuits controlling mitochondrial biogenesis and function | Q35683727 | ||
| Cardiac dysfunction in the R6/2 mouse model of Huntington's disease | Q35745626 | ||
| Aging-associated reductions in AMP-activated protein kinase activity and mitochondrial biogenesis | Q35830162 | ||
| Oxidative damage in Huntington's disease pathogenesis | Q36620593 | ||
| Age‐Dependent Striatal Excitotoxic Lesions Produced by the Endogenous Mitochondrial Inhibitor Malonate | Q36696707 | ||
| Transcriptional signatures in Huntington's disease | Q36752799 | ||
| 3-Nitropropionic acid-exogenous animal neurotoxin and possible human striatal toxin. | Q36934346 | ||
| Huntingtin modulates transcription, occupies gene promoters in vivo, and binds directly to DNA in a polyglutamine-dependent manner | Q36981055 | ||
| RETRACTED: Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype | Q37036533 | ||
| The gene coding for PGC-1alpha modifies age at onset in Huntington's Disease | Q37071213 | ||
| Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models | Q37112122 | ||
| Whole body overexpression of PGC-1alpha has opposite effects on hepatic and muscle insulin sensitivity | Q37162234 | ||
| Sensitivity of lipid metabolism and insulin signaling to genetic alterations in hepatic peroxisome proliferator-activated receptor-gamma coactivator-1alpha expression | Q37236343 | ||
| Impaired PGC-1alpha function in muscle in Huntington's disease. | Q37323316 | ||
| GCN5-mediated transcriptional control of the metabolic coactivator PGC-1beta through lysine acetylation | Q37338983 | ||
| Mitochondrial structural and functional dynamics in Huntington's disease | Q37356257 | ||
| PGC-1alpha, SIRT1 and AMPK, an energy sensing network that controls energy expenditure | Q37412140 | ||
| Peroxisome proliferator-activated receptor gamma coactivator-1 promotes cardiac mitochondrial biogenesis | Q37526796 | ||
| Nutrient-dependent regulation of PGC-1alpha's acetylation state and metabolic function through the enzymatic activities of Sirt1/GCN5 | Q37652237 | ||
| Slowed progression in models of Huntington disease by adipose stem cell transplantation | Q39770706 | ||
| Depletion of CBP is directly linked with cellular toxicity caused by mutant huntingtin | Q40269204 | ||
| Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release | Q40551652 | ||
| Bioenergetic analysis of peroxisome proliferator-activated receptor gamma coactivators 1alpha and 1beta (PGC-1alpha and PGC-1beta) in muscle cells | Q40649634 | ||
| Cell death triggered by polyglutamine-expanded huntingtin in a neuronal cell line is associated with degradation of CREB-binding protein | Q40682460 | ||
| Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. | Q40721588 | ||
| Mitochondrial DNA background modifies the bioenergetics of NARP/MILS ATP6 mutant cells | Q42010130 | ||
| Rosiglitazone treatment prevents mitochondrial dysfunction in mutant huntingtin-expressing cells: possible role of peroxisome proliferator-activated receptor-gamma (PPARgamma) in the pathogenesis of Huntington disease | Q42434605 | ||
| Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia. | Q42438521 | ||
| Biochemical adaptation in the skeletal muscle of rats depleted of creatine with the substrate analogue beta-guanidinopropionic acid | Q42866964 | ||
| PGC-1alpha as modifier of onset age in Huntington disease | Q42927080 | ||
| Metabolic network abnormalities in early Huntington's disease: an [(18)F]FDG PET study. | Q43790732 | ||
| Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain | Q44092333 | ||
| The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis. | Q44376761 | ||
| Endogenous mitochondrial oxidative stress: neurodegeneration, proteomic analysis, specific respiratory chain defects, and efficacious antioxidant therapy in superoxide dismutase 2 null mice | Q44726528 | ||
| Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice | Q44931209 | ||
| Progressive abnormalities in skeletal muscle and neuromuscular junctions of transgenic mice expressing the Huntington's disease mutation | Q45173078 | ||
| Mitochondrial impairment in patients and asymptomatic mutation carriers of Huntington's disease | Q45259959 | ||
| The metabolic profile of early Huntington's disease--a combined human and transgenic mouse study | Q45288758 | ||
| Beneficial effects of rolipram in the R6/2 mouse model of Huntington's disease | Q45289413 | ||
| Striatal glucose consumption in chorea-free subjects at risk of Huntington's disease | Q45289936 | ||
| Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy | Q45290303 | ||
| Serial changes of cerebral glucose metabolism and caudate size in persons at risk for Huntington's disease. | Q45291612 | ||
| Mitochondrial defect in Huntington's disease caudate nucleus | Q45291734 | ||
| Phosphodiesterase type IV inhibition prevents sequestration of CREB binding protein, protects striatal parvalbumin interneurons and rescues motor deficits in the R6/2 mouse model of Huntington's disease | Q45293113 | ||
| Energy metabolism defects in Huntington's disease and effects of coenzyme Q10. | Q45293805 | ||
| Biochemical abnormalities and excitotoxicity in Huntington's disease brain. | Q45297145 | ||
| Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin | Q45297269 | ||
| Formation of polyglutamine inclusions in non-CNS tissue | Q45298039 | ||
| HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism | Q45298206 | ||
| Higher sedentary energy expenditure in patients with Huntington's disease | Q45299677 | ||
| Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG. | Q45299923 | ||
| Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice | Q45299927 | ||
| Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy | Q45300771 | ||
| 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 | ||
| Metformin therapy in a transgenic mouse model of Huntington's disease. | Q45303200 | ||
| Myopathy as a first symptom of Huntington's disease in a Marathon runner | Q45305237 | ||
| Clinical correlates of mitochondrial function in Huntington's disease muscle | Q45305337 | ||
| Weight loss in early stage of Huntington's disease | Q45307146 | ||
| Muscle-specific differences in the response of mitochondrial proteins to beta-GPA feeding: an evaluation of potential mechanisms | Q46076268 | ||
| Age-dependent vulnerability of the striatum to the mitochondrial toxin 3-nitropropionic acid. | Q48365871 | ||
| Experimental depletion of creatine and phosphocreatine from skeletal muscle | Q69738777 | ||
| Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity | Q95721056 | ||
| P433 | issue | 16 | |
| P921 | main subject | Huntington's disease | Q190564 |
| steatosis | Q1365091 | ||
| P304 | page(s) | 3190-3205 | |
| P577 | publication date | 2010-06-07 | |
| P1433 | published in | Human Molecular Genetics | Q2720965 |
| P1476 | title | Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation | |
| P478 | volume | 19 |
| Q90721199 | A Role for PGC-1α in Transcription and Excitability of Neocortical and Hippocampal Excitatory Neurons |
| Q45290293 | A study of molecular changes relating to energy metabolism and cellular stress in people with Huntington's disease: looking for biomarkers |
| Q38539535 | AMPK-mediated regulation of neuronal metabolism and function in brain diseases |
| Q89620018 | Age-Dependent Decline in Cardiac Function in Guanidinoacetate-N-Methyltransferase Knockout Mice |
| Q37964359 | Antioxidants in Huntington's disease |
| Q41662736 | Are Astrocytes the Predominant Cell Type for Activation of Nrf2 in Aging and Neurodegeneration? |
| Q58584663 | Bioenergetics in fibroblasts of patients with Huntington disease are associated with age at onset |
| Q52309831 | Cardiac mTORC1 Dysregulation Impacts Stress Adaptation and Survival in Huntington's Disease. |
| Q50421506 | Cell-specific deletion of PGC-1α from medium spiny neurons causes transcriptional alterations and age-related motor impairment |
| Q30613057 | Cerebellar transcriptional alterations with Purkinje cell dysfunction and loss in mice lacking PGC-1α. |
| Q33729341 | Comparison of Sirtuin 3 Levels in ALS and Huntington's Disease-Differential Effects in Human Tissue Samples vs. Transgenic Mouse Models |
| Q34391101 | Developmental alterations in motor coordination and medium spiny neuron markers in mice lacking pgc-1α |
| Q34431341 | Downregulation of genes involved in metabolism and oxidative stress in the peripheral leukocytes of Huntington's disease patients |
| Q39335116 | Early transcriptional changes linked to naturally occurring Huntington's disease mutations in neural derivatives of human embryonic stem cells. |
| Q33729818 | Effects of creatine and β-guanidinopropionic acid and alterations in creatine transporter and creatine kinases expression in acute seizure and chronic epilepsy models |
| Q26795417 | Electron Transport Disturbances and Neurodegeneration: From Albert Szent-Györgyi's Concept (Szeged) till Novel Approaches to Boost Mitochondrial Bioenergetics |
| Q28396785 | Emerging roles of Nrf2 and phase II antioxidant enzymes in neuroprotection |
| Q38013005 | Energy dysfunction in Huntington's disease: insights from PGC-1α, AMPK, and CKB. |
| Q37368003 | Enhanced mitochondrial biogenesis ameliorates disease phenotype in a full-length mouse model of Huntington's disease |
| Q36301359 | Ethosuximide Induces Hippocampal Neurogenesis and Reverses Cognitive Deficits in an Amyloid-β Toxin-induced Alzheimer Rat Model via the Phosphatidylinositol 3-Kinase (PI3K)/Akt/Wnt/β-Catenin Pathway |
| Q34657923 | Extracts of adipose derived stem cells slows progression in the R6/2 model of Huntington's disease |
| Q90478664 | Fat Therapeutics: The Clinical Capacity of Adipose-Derived Stem Cells and Exosomes for Human Disease and Tissue Regeneration |
| Q45305356 | HACE1 is essential for astrocyte mitochondrial function and influences Huntington disease phenotypes in vivo. |
| Q28386493 | HdhQ111 Mice Exhibit Tissue Specific Metabolite Profiles that Include Striatal Lipid Accumulation |
| Q45301958 | Hugging tight in Huntington's |
| Q39035495 | Huntington's Disease: Mechanisms of Pathogenesis and Therapeutic Strategies |
| Q37889820 | Huntington's disease, calcium, and mitochondria |
| Q29248581 | Huntington’s disease blood and brain show a common gene expression pattern and share an immune signature with Alzheimer’s disease |
| Q42703671 | Inhibitory Effects of Bisphenol-A on Neural Stem Cells Proliferation and Differentiation in the Rat Brain Are Dependent on Wnt/β-Catenin Pathway |
| Q90310135 | Long noncoding RNA lncARSR promotes nonalcoholic fatty liver disease and hepatocellular carcinoma by promoting YAP1 and activating the IRS2/AKT pathway |
| Q28394301 | Metabolism in HD: still a relevant mechanism? |
| Q33799027 | Mice lacking the transcriptional coactivator PGC-1α exhibit alterations in inhibitory synaptic transmission in the motor cortex. |
| Q39016389 | Mitochondrial Dysfunction and Biogenesis in Neurodegenerative diseases: Pathogenesis and Treatment. |
| Q28387647 | Mitochondrial dysfunction in neurodegenerative diseases |
| Q35086831 | Molecular characterization of skeletal muscle atrophy in the R6/2 mouse model of Huntington's disease |
| Q34944325 | Mutual exacerbation of peroxisome proliferator-activated receptor γ coactivator 1α deregulation and α-synuclein oligomerization |
| Q38167610 | Natural product-derived pharmacological modulators of Nrf2/ARE pathway for chronic diseases. |
| Q37963878 | Neuroprotective Mechanisms of PPARδ: Modulation of Oxidative Stress and Inflammatory Processes |
| Q34215149 | Neuroprotective and metabolic effects of resveratrol: therapeutic implications for Huntington's disease and other neurodegenerative disorders |
| Q36813115 | Neuroprotective effects of PPAR-γ agonist rosiglitazone in N171-82Q mouse model of Huntington's disease |
| Q42097305 | Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets |
| Q35052819 | PGC-1alpha downstream transcription factors NRF-1 and TFAM are genetic modifiers of Huntington disease. |
| Q28575407 | PGC-1α negatively regulates extrasynaptic NMDAR activity and excitotoxicity |
| Q30574513 | PGC-1α overexpression exacerbates β-amyloid and tau deposition in a transgenic mouse model of Alzheimer's disease |
| Q27001671 | PGC-1α, mitochondrial dysfunction, and Huntington's disease |
| Q42792348 | Peroxisome-proliferator-activated receptor gamma coactivator 1 α contributes to dysmyelination in experimental models of Huntington's disease |
| Q35750228 | Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease |
| Q38191477 | Prospects for neuroprotective therapies in prodromal Huntington's disease |
| Q55414200 | RNA Aptamers Rescue Mitochondrial Dysfunction in a Yeast Model of Huntington’s Disease. |
| Q28386202 | Role of oxidative DNA damage in mitochondrial dysfunction and Huntington's disease pathogenesis |
| Q34443537 | Roles of resveratrol and other grape-derived polyphenols in Alzheimer's disease prevention and treatment |
| Q41863025 | SPBP is a sulforaphane induced transcriptional coactivator of NRF2 regulating expression of the autophagy receptor p62/SQSTM1. |
| Q37530250 | Structural and molecular myelination deficits occur prior to neuronal loss in the YAC128 and BACHD models of Huntington disease |
| Q26822632 | The effect of the creatine analogue beta-guanidinopropionic acid on energy metabolism: a systematic review |
| Q49823902 | The mitochondrial transcription factor TFAM in neurodegeneration: emerging evidence and mechanisms |
| Q47152282 | Transcriptional regulators of redox balance and other homeostatic processes with the potential to alter neurodegenerative disease trajectory |
| Q36379946 | Transducer of regulated CREB-binding proteins (TORCs) transcription and function is impaired in Huntington's disease |
| Q38607117 | Treating the whole body in Huntington's disease |
| Q34075646 | Two-point magnitude MRI for rapid mapping of brown adipose tissue and its application to the R6/2 mouse model of Huntington disease |
| Q45302304 | mRNA expression levels of PGC-1α in a transgenic and a toxin model of Huntington's disease. |
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