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Energy deficit in Huntington disease: why it matters
scientific article published on February 2011
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scholarly article
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
review article
1 reference
stated in
Europe PubMed Central
title
Energy deficit in Huntington disease: why it matters
(English)
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
main subject
Huntington's disease
0 references
author name string
Fanny Mochel
series ordinal
1
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
Ronald G Haller
series ordinal
2
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
language of work or name
English
0 references
publication date
1 February 2011
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
published in
Journal of Clinical Investigation
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
volume
121
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
issue
2
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
page(s)
493-499
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
cites work
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6 September 2017
Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolism
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Selective defect of in vivo glycolysis in early Huntington's disease striatum
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6 September 2017
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6 September 2017
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6 September 2017
Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice
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6 September 2017
Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro
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6 September 2017
Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules
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6 September 2017
The energetics of Huntington's disease
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6 September 2017
Huntingtin-protein interactions and the pathogenesis of Huntington's disease
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6 September 2017
The hunt for huntingtin function: interaction partners tell many different stories
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6 September 2017
Transcriptional abnormalities in Huntington disease
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6 September 2017
A mitochondrial paradigm for degenerative diseases and ageing
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6 September 2017
Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo
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6 September 2017
Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles
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6 September 2017
Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice
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6 September 2017
The selective vulnerability of striatopallidal neurons
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6 September 2017
Huntingtin interacts with a family of WW domain proteins
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6 September 2017
Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion
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6 September 2017
Striatal glucose metabolism and dopamine D2 receptor binding in asymptomatic gene carriers and patients with Huntington's disease
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6 September 2017
Differential effects of creatine depletion on the regulation of enzyme activities and on creatine-stimulated mitochondrial respiration in skeletal muscle, heart, and brain
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6 September 2017
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.
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6 September 2017
Characterization and localization of the Huntington disease gene product
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6 September 2017
Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes
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6 September 2017
Superoxide radical and iron modulate aconitase activity in mammalian cells
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6 September 2017
Dilated cardiomyopathy and neonatal lethality in mutant mice lacking manganese superoxide dismutase
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6 September 2017
A polymorphic DNA marker genetically linked to Huntington's disease
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6 September 2017
Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis
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14 September 2017
Slowed progression in models of Huntington disease by adipose stem cell transplantation
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14 September 2017
Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington disease
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14 September 2017
Increased oxidative damage and mitochondrial abnormalities in the peripheral blood of Huntington's disease patients
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14 September 2017
Extended polyglutamine repeats trigger a feedback loop involving the mitochondrial complex III, the proteasome and huntingtin aggregates
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14 September 2017
Neurochemical changes in Huntington R6/2 mouse striatum detected by in vivo 1H NMR spectroscopy
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14 September 2017
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release
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14 September 2017
Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice
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14 September 2017
Body weight, feeding, and drinking behaviors in rats with kainic acid-induced lesions of striatal neurons—With a note on body weight symptomatology in Huntington's disease
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14 September 2017
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines.
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PubMed Central
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28 September 2017
Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription
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PubMed Central
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28 September 2017
Wild type Huntingtin reduces the cellular toxicity of mutant Huntingtin in mammalian cell models of Huntington's disease
1 reference
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PubMed Central
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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
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28 September 2017
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
1 reference
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PubMed Central
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23 June 2018
Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production
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PubMed Central
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23 June 2018
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
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PubMed Central
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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
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23 June 2018
Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse.
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PubMed Central
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23 June 2018
Oxidative stress in Huntington's disease
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PubMed Central
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23 June 2018
Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.
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23 June 2018
Chronic 3-nitropropionic acid treatment in baboons replicates the cognitive and motor deficits of Huntington's disease
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PubMed Central
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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
23 June 2018
Body weight and dietary factors in Huntington's disease patients compared with matched controls
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
23 June 2018
Biochemical abnormalities in Huntington's chorea brains
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
23 June 2018
Factors associated with slow progression in Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
23 June 2018
Serial changes of cerebral glucose metabolism and caudate size in persons at risk for Huntington's disease.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
23 June 2018
Resveratrol protects against peripheral deficits in a mouse model of Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Dietary anaplerotic therapy improves peripheral tissue energy metabolism in patients with Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
PGC-1alpha as modifier of onset age in Huntington disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Proteomic and oxidative stress analysis in human brain samples of Huntington disease.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
The metabolic profile of early Huntington's disease--a combined human and transgenic mouse study
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Huntingtin-deficient zebrafish exhibit defects in iron utilization and development.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: effect of histone deacetylase inhibitors.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Cytotoxicity of a mutant huntingtin fragment in yeast involves early alterations in mitochondrial OXPHOS complexes II and III.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Gene expression in Huntington's disease skeletal muscle: a potential biomarker
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
High-dose creatine therapy for Huntington disease: a 2-year clinical and MRS study.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Huntingtin phosphorylation on serine 421 is significantly reduced in the striatum and by polyglutamine expansion in vivo
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Mitochondrial impairment in patients and asymptomatic mutation carriers of Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Circulating and cerebrospinal fluid ghrelin and leptin: potential role in altered body weight in Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Assessment of the nutrition status of patients with Huntington's disease.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Creatine supplementation in Huntington's disease: a placebo-controlled pilot trial
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Ascorbate treatment attenuates the Huntington behavioral phenotype in mice
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Increased survival and neuroprotective effects of BN82451 in a transgenic mouse model of Huntington's disease.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Weight loss in early stage of Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Comparative analysis of superoxide dismutase activity between acute pharmacological models and a transgenic mouse model of Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
3-nitropropionic acid induced in vivo protein oxidation in striatal and cortical synaptosomes: insights into Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Higher sedentary energy expenditure in patients with Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Biochemical abnormalities and excitotoxicity in Huntington's disease brain.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
1H NMR spectroscopy studies of Huntington's disease: correlations with CAG repeat numbers
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Partial inhibition of brain succinate dehydrogenase by 3-nitropropionic acid is sufficient to initiate striatal degeneration in rat.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Energy metabolism defects in Huntington's disease and effects of coenzyme Q10.
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Mitochondrial defect in Huntington's disease caudate nucleus
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Striatal glucose consumption in chorea-free subjects at risk of Huntington's disease
1 reference
stated in
PubMed Central
reference URL
https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3026743
retrieved
1 September 2018
Identifiers
DOI
10.1172/JCI45691
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
PMCID
3026743
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
PubMed ID
21285522
1 reference
stated in
Europe PubMed Central
PMCID
3026743
retrieved
24 August 2017
ResearchGate publication ID
49802425
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