Novel Metabolic Abnormalities in the Tricarboxylic Acid Cycle in Peripheral Cells From Huntington's Disease Patients (Q36127832)

12 August 2017

Novel Metabolic Abnormalities in the Tricarboxylic Acid Cycle in Peripheral Cells From Huntington's Disease Patients (English)

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12 August 2017

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Nima N Naseri

1

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12 August 2017

Joseph Bonica

2

1 reference

12 August 2017

Hui Xu

3

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12 August 2017

Larry C Park

4

1 reference

12 August 2017

Jamshid Arjomand

5

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12 August 2017

Zhengming Chen

6

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12 August 2017

Gary E Gibson

7

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12 August 2017

language of work or name

English

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publication date

9 September 2016

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12 August 2017

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12 August 2017

11

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12 August 2017

9

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12 August 2017

e0160384

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Creative Commons Attribution 4.0 International

12 August 2017

start time

9 September 2016

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April 2022 Public Data File from Crossref

copyright status

copyrighted

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cites work

Abnormalities in the tricarboxylic Acid cycle in Huntington disease and in a Huntington disease mouse model

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The incidence and prevalence of Huntington's disease: a systematic review and meta-analysis

2 October 2017

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Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease

2 October 2017

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Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage

2 October 2017

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Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli

2 October 2017

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Mice deficient in dihydrolipoyl succinyl transferase show increased vulnerability to mitochondrial toxins

2 October 2017

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

2 October 2017

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Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity

2 October 2017

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Regulation of pyruvate dehydrogenase complex activity by reversible phosphorylation

2 October 2017

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The alpha-ketoglutarate dehydrogenase complex in neurodegeneration

2 October 2017

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Transglutaminase-catalyzed inactivation of glyceraldehyde 3-phosphate dehydrogenase and alpha-ketoglutarate dehydrogenase complex by polyglutamine domains of pathological length

2 October 2017

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

2 October 2017

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Age‐Dependent Striatal Excitotoxic Lesions Produced by the Endogenous Mitochondrial Inhibitor Malonate

2 October 2017

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Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

2 October 2017

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

2 October 2017

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A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding

2 October 2017

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Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse.

2 October 2017

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

2 July 2018

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Patterns of cerebral glucose utilization in Parkinson's disease and Huntington's disease

2 July 2018

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Decreased pyruvate dehydrogenase complex activity in Huntington and Alzheimer brain

2 July 2018

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Cerebral metabolism and atrophy in huntington's disease determined by18FDG and computed tomographic scan

2 July 2018

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Local cerebral glucose utilization in symptomatic and presymptomatic Huntington's disease

2 July 2018

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Distribution of phosphate-activated glutaminase, succinic dehydrogenase, pyruvate dehydrogenase and gamma-glutamyl transpeptidase in post-mortem brain from Huntington's disease and agonal cases

2 July 2018

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Observing Huntington's disease: the European Huntington's Disease Network's REGISTRY

2 July 2018

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Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5017661

Statistical models in assessing fold change of gene expression in real-time RT-PCR experiments

23 September 2018

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Mitochondrial abnormalities in Alzheimer brain: mechanistic implications

23 September 2018

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Tricarboxylic acid cycle enzymes following thiamine deficiency

23 September 2018

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Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease

23 September 2018

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Rapid aggregate formation of the huntingtin N-terminal fragment carrying an expanded polyglutamine tract

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5017661

Biochemical abnormalities and excitotoxicity in Huntington's disease brain.

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5017661

Mitochondrial defect in Huntington's disease caudate nucleus

23 September 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5017661

Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy

23 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5017661

Inhibition of α-ketoglutarate-and pyruvate dehydrogenase complexes in E. coli by a glutathione S-transferase containing a pathological length poly-Q domain: A possible role of energy deficit in neurological diseases associated with poly-Q expansions

23 September 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=5017661

Unified Huntington's disease rating scale: Reliability and consistency

21 October 2018

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Crossref

https://api.crossref.org/works/10.1371/JOURNAL.PONE.0160384

Correction of hemophilia B in canine and murine models using recombinant adeno-associated viral vectors

21 January 2018

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Crossref

https://api.crossref.org/works/10.1371/JOURNAL.PONE.0160384

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

21 January 2018

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12 December 2020

Inhibition of N-acetylaspartate production: implications for 1H MRS studies in vivo

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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

The regeneration of reduced glutathione in rat forebrain mitochondria identifies metabolic pathways providing the NADPH required

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12 December 2020

Mitochondrial enzyme deficiencies in Down's syndrome

1 reference

12 December 2020

10.1371/JOURNAL.PONE.0160384

Identifiers

DOI

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12 August 2017

0 references

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12 August 2017

PubMed publication ID

27611087

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