(Q37750542)

22 August 2017

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title

Mitochondrial functional alterations in relation to pathophysiology of Huntington's disease (English)

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22 August 2017

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Mritunjay Pandey

1

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22 August 2017

Kochupurackal P Mohanakumar

series ordinal

2

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22 August 2017

Rajamma Usha

series ordinal

3

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22 August 2017

publication date

1 June 2010

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Journal of Bioenergetics and Biomembranes

22 August 2017

published in

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22 August 2017

volume

42

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22 August 2017

issue

3

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22 August 2017

page(s)

217-226

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Relationships among molecular genetic and respiratory properties of Parkinson's disease cybrid cells show similarities to Parkinson's brain tissues

22 August 2017

cites work

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Crossref

https://api.crossref.org/works/10.1007%2FS10863-010-9288-5

Impaired mitochondrial trafficking in Huntington's disease

21 January 2018

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Crossref

https://api.crossref.org/works/10.1007%2FS10863-010-9288-5

Evidence for a defect in NADH: ubiquinone oxidoreductase (complex I) in Huntington's disease

21 January 2018

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Crossref

https://api.crossref.org/works/10.1007%2FS10863-010-9288-5

The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis.

21 January 2018

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12 December 2020

Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin

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12 December 2020

Loss of mitochondrial membrane potential is associated with increase in mitochondrial volume: physiological role in neurones.

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12 December 2020

Extended polyglutamine repeats trigger a feedback loop involving the mitochondrial complex III, the proteasome and huntingtin aggregates

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12 December 2020

Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines.

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12 December 2020

Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins

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12 December 2020

Mitochondrial DNA damage is a hallmark of chemically induced and the R6/2 transgenic model of Huntington's disease

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12 December 2020

Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons

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12 December 2020

Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration

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12 December 2020

Striatal dopamine level contributes to hydroxyl radical generation and subsequent neurodegeneration in the striatum in 3-nitropropionic acid-induced Huntington's disease in rats

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12 December 2020

Biochemical abnormalities and excitotoxicity in Huntington's disease brain.

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12 December 2020

Ca(2+) signalling in mitochondria: mechanism and role in physiology and pathology.

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12 December 2020

Mitochondrial structural and functional dynamics in Huntington's disease

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12 December 2020

Oxidative stress promotes mutant huntingtin aggregation and mutant huntingtin-dependent cell death by mimicking proteasomal malfunction.

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12 December 2020

Characterization of cybrid cell lines containing mtDNA from Huntington's disease patients.

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12 December 2020

Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington disease

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12 December 2020

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.

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12 December 2020

Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG.

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12 December 2020

Reduced axonal transport in Parkinson's disease cybrid neurites is restored by light therapy

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12 December 2020

Regulation of mitochondrial matrix volume

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12 December 2020

Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization

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12 December 2020

Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA.

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12 December 2020

Impaired PGC-1alpha function in muscle in Huntington's disease.

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12 December 2020

Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release

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12 December 2020

Oxidative stress in skin fibroblasts cultures of patients with Huntington's disease.

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12 December 2020

Proteomic and oxidative stress analysis in human brain samples of Huntington disease.

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12 December 2020

Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease

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12 December 2020

Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid

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12 December 2020

Differential contributions of Caenorhabditis elegans histone deacetylases to huntingtin polyglutamine toxicity.

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12 December 2020

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

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12 December 2020

Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus

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12 December 2020

Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues

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12 December 2020

Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates

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12 December 2020

Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin

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12 December 2020

Mitochondrial-dependent apoptosis in Huntington's disease human cybrids.

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12 December 2020

Caspase-dependent and -independent cell death induced by 3-nitropropionic acid in rat cortical neurons.

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12 December 2020

Mitochondrial defect in Huntington's disease caudate nucleus

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12 December 2020

Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in Drosophila

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12 December 2020

Reactive oxygen species and the central nervous system

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12 December 2020

Duplication of biochemical changes of Huntington's chorea by intrastriatal injections of glutamic and kainic acids

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12 December 2020

Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia.

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12 December 2020

Regional mitochondrial respiratory activity in Huntington's disease brain

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12 December 2020

A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice

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12 December 2020

Complex I defect in muscle from patients with Huntington's disease.

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12 December 2020

Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction.

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12 December 2020

In vivo calpain/caspase cross-talk during 3-nitropropionic acid-induced striatal degeneration: implication of a calpain-mediated cleavage of active caspase-3.

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12 December 2020

Mitochondrial cyclic AMP response element-binding protein (CREB) mediates mitochondrial gene expression and neuronal survival

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12 December 2020

Coenzyme Q10 serum levels in Huntington's disease

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12 December 2020

Huntington's disease, energy, and excitotoxicity

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12 December 2020

Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity

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12 December 2020

Striatal modulation of cAMP-response-element-binding protein (CREB) after excitotoxic lesions: implications with neuronal vulnerability in Huntington's disease.

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12 December 2020

Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid

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12 December 2020

Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice

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12 December 2020

Oxidative damage to mitochondrial DNA in Huntington's disease parietal cortex

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12 December 2020

Calpain activation in Huntington's disease.

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12 December 2020

Metabolic changes in the basal ganglia of patients with Huntington's disease: an in situ hybridization study of cytochrome oxidase subunit I mRNA.

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12 December 2020

Energy metabolism defects in Huntington's disease and effects of coenzyme Q10.

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12 December 2020

Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing

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12 December 2020

Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences

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12 December 2020

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.

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12 December 2020

Mitochondrial swelling impairs the transport of organelles in cerebellar granule neurons

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12 December 2020

Neuropathological classification of Huntington's disease

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12 December 2020

Mitochondrial aconitase is a source of hydroxyl radical. An electron spin resonance investigation

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12 December 2020

L-DOPA-induced 6-hydroxydopamine production in the striata of rodents is sensitive to the degree of denervation

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12 December 2020

Calpain is a major cell death effector in selective striatal degeneration induced in vivo by 3-nitropropionate: implications for Huntington's disease.

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12 December 2020

Peroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulator

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12 December 2020

Towards a transgenic model of Huntington's disease in a non-human primate

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12 December 2020

Sustained elevation of extracellular dopamine causes motor dysfunction and selective degeneration of striatal GABAergic neurons.

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12 December 2020

Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release

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12 December 2020

A cellular model that recapitulates major pathogenic steps of Huntington's disease

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12 December 2020

Dopamine determines the vulnerability of striatal neurons to the N-terminal fragment of mutant huntingtin through the regulation of mitochondrial complex II.

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12 December 2020

Selective defect of in vivo glycolysis in early Huntington's disease striatum

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12 December 2020

Expression of the Huntington's disease gene is regulated in astrocytes in the arcuate nucleus of the hypothalamus of postpartum rats

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12 December 2020

Functional anatomy of the basal ganglia. I. The cortico-basal ganglia-thalamo-cortical loop

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12 December 2020

Glutamate transporters in neurologic disease

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12 December 2020

Complex II inhibition by 3-NP causes mitochondrial fragmentation and neuronal cell death via an NMDA- and ROS-dependent pathway.

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12 December 2020

A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.

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12 December 2020

Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production

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12 December 2020

Dopamine modulates the susceptibility of striatal neurons to 3-nitropropionic acid in the rat model of Huntington's disease.

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12 December 2020

Huntingtin promotes cell survival by preventing Pak2 cleavage

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12 December 2020

Unraveling a role for dopamine in Huntington's disease: the dual role of reactive oxygen species and D2 receptor stimulation

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12 December 2020

Clinical correlates of mitochondrial function in Huntington's disease muscle

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12 December 2020

Mitochondrial calcium, oxidative stress and apoptosis in a neurodegenerative disease model induced by 3-nitropropionic acid

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12 December 2020

Apoptotic mode of cell death in substantia nigra following intranigral infusion of the parkinsonian neurotoxin, MPP+ in Sprague-Dawley rats: cellular, molecular and ultrastructural evidences

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12 December 2020

DNA End Labeling (TUNEL) in Huntington's Disease and Other Neuropathological Conditions

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12 December 2020

Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats

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12 December 2020

Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration.

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12 December 2020

Cytochrome c oxidase isoform IV-2 is involved in 3-nitropropionic acid-induced toxicity in striatal astrocytes

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12 December 2020

Huntingtin protein colocalizes with lesions of neurodegenerative diseases: An investigation in Huntington's, Alzheimer's, and Pick's diseases

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12 December 2020

Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutation

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12 December 2020

Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease

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12 December 2020

Mitochondrial sensitivity and altered calcium handling underlie enhanced NMDA-induced apoptosis in YAC128 model of Huntington's disease.

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12 December 2020

Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: effect of histone deacetylase inhibitors.

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12 December 2020

Inhibitors of cytochrome c release with therapeutic potential for Huntington's disease

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12 December 2020

N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking

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12 December 2020

Mitochondrial dysfunction in Huntington's disease: the bioenergetics of isolated and in situ mitochondria from transgenic mice

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12 December 2020

Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease

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12 December 2020

Ubiquitin-proteasome system alterations in a striatal cell model of Huntington's disease

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12 December 2020

In situ mitochondrial Ca2+ buffering differences of intact neurons and astrocytes from cortex and striatum.

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12 December 2020

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin

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12 December 2020

Cytochrome C and caspase-9 expression in Huntington's disease

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12 December 2020

Increased oxidative damage and mitochondrial abnormalities in the peripheral blood of Huntington's disease patients

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12 December 2020

Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro

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12 December 2020

Effect of coenzyme Q10 and vitamin E on brain energy metabolism in the animal model of Huntington's disease

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12 December 2020

Oxidative stress parameters in plasma of Huntington's disease patients, asymptomatic Huntington's disease gene carriers and healthy subjects : a cross-sectional study

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12 December 2020

Effects of overexpression of huntingtin proteins on mitochondrial integrity

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12 December 2020

Chronic, low-dose rotenone reproduces Lewy neurites found in early stages of Parkinson's disease, reduces mitochondrial movement and slowly kills differentiated SH-SY5Y neural cells

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12 December 2020

10.1007/S10863-010-9288-5

Identifiers

DOI

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22 August 2017

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