PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function. (Q30583566)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22786682%20AND%20SRC:MED&resulttype=core&format=json

7 February 2020

title

PGC-1α rescues Huntington's disease proteotoxicity by preventing oxidative stress and promoting TFEB function (English)

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7 February 2020

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2

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7 February 2020

Bradley E Morrison

3

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7 February 2020

author name string

Taiji Tsunemi

1

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7 February 2020

Kathryn R Soriano

4

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7 February 2020

Jonathan Au

5

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7 February 2020

Ruben A Vázquez Roque

6

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7 February 2020

Eduardo R Lazarowski

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7 February 2020

Vincent A Damian

8

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7 February 2020

Albert R La Spada

10

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7 February 2020

Eliezer Masliah

9

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7 February 2020

language of work or name

English

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publication date

1 July 2012

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Science Translational Medicine

7 February 2020

published in

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7 February 2020

volume

4

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7 February 2020

issue

142

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7 February 2020

page(s)

142ra97

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TFEB links autophagy to lysosomal biogenesis

7 February 2020

cites work

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

PARIS (ZNF746) repression of PGC-1α contributes to neurodegeneration in Parkinson's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

PGC-1α, a potential therapeutic target for early intervention in Parkinson's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Mitochondrial autophagy in neural function, neurodegenerative disease, neuron cell death, and aging

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Oral N-acetyl-cysteine attenuates loss of dopaminergic terminals in alpha-synuclein overexpressing mice

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Autophagy gone awry in neurodegenerative diseases

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Neurodegeneration in a transgenic mouse model of multiple system atrophy is associated with altered expression of oligodendroglial-derived neurotrophic factors

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Repeat expansion disease: progress and puzzles in disease pathogenesis

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

A gene network regulating lysosomal biogenesis and function

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Oxidative modifications, mitochondrial dysfunction, and impaired protein degradation in Parkinson's disease: how neurons are lost in the Bermuda triangle

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Fibril specific, conformation dependent antibodies recognize a generic epitope common to amyloid fibrils and fibrillar oligomers that is absent in prefibrillar oligomers

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

The roles of intracellular protein-degradation pathways in neurodegeneration

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Conditional and inducible transgene expression in mice through the combinatorial use of Cre-mediated recombination and tetracycline induction.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

PGC-1alpha deficiency causes multi-system energy metabolic derangements: muscle dysfunction, abnormal weight control and hepatic steatosis

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Experimental therapeutics in transgenic mouse models of Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

The energetics of Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Transcriptional regulatory circuits controlling mitochondrial biogenesis and function

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Toxic proteins in neurodegenerative disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Spinocerebellar ataxias due to mitochondrial defects

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Mouse models of Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Pathogenesis of primary defects in mitochondrial ATP synthesis.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Complex trait analysis of the mouse striatum: independent QTLs modulate volume and neuron number

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Oxidative stress in Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

A cold-inducible coactivator of nuclear receptors linked to adaptive thermogenesis

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Protamine-Cre recombinase transgenes efficiently recombine target sequences in the male germ line of mice, but not in embryonic stem cells

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Comparison of two quantitative methods for the evaluation of neuronal number in the frontal cortex in Alzheimer disease.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Clinical aspects of CAG repeat diseases

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases?

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Neuropathological classification of Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Weight loss in Huntington disease increases with higher CAG repeat number

20 June 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3003799

HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3003799

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3003799

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3003799

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3003799

Huntington's disease--making connections.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3003799

Cardiac-specific induction of the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator-1alpha promotes mitochondrial biogenesis and reversible cardiomyopathy in a developmental stage-dependent manner

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3003799

Hsp70 and Hsp40 attenuate formation of spherical and annular polyglutamine oligomers by partitioning monomer

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3003799

Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3003799

Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70.

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3003799

Oxidative modification and inactivation of the proteasome during coronary occlusion/reperfusion

21 January 2018

1 reference

https://api.crossref.org/works/10.1126%2FSCITRANSLMED.3003799

Polyglutamine-expanded ataxin-7 promotes non-cell-autonomous purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice.

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice.

26 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4096245

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

26 November 2018

1 reference

12 December 2020

A stereological study of MRI and the Cavalieri principle combined for diagnosis and monitoring of brain tumor volume

1 reference

12 December 2020

4-Hydroxy-2-nonenal-mediated impairment of intracellular proteolysis during oxidative stress. Identification of proteasomes as target molecules

1 reference

12 December 2020

Modification of glucose-6-phosphate dehydrogenase by 4-hydroxy-2-nonenal. Formation of cross-linked protein that inhibits the multicatalytic protease

1 reference

12 December 2020

10.1126/SCITRANSLMED.3003799

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22786682%20AND%20SRC:MED&resulttype=core&format=json

7 February 2020

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22786682%20AND%20SRC:MED&resulttype=core&format=json

7 February 2020

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22786682%20AND%20SRC:MED&resulttype=core&format=json