Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells (Q40290417)

19 September 2017

Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells (English)

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19 September 2017

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Zhengkuan Mao

1

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19 September 2017

Yeun Su Choo

series ordinal

2

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19 September 2017

Mathieu Lesort

series ordinal

3

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19 September 2017

language of work or name

English

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publication date

1 April 2006

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European Journal of Neuroscience

19 September 2017

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19 September 2017

23

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19 September 2017

7

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19 September 2017

1701-1710

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Identification of combinatorial drug regimens for treatment of Huntington's disease using Drosophila.

19 September 2017

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

3-Nitropropionate, the toxic substance of Indigofera , is a suicide inactivator of succinate dehydrogenase

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Tissue transglutaminase contributes to disease progression in the R6/2 Huntington's disease mouse model via aggregate-independent mechanisms

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Huntington's disease, energy, and excitotoxicity

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Mitochondria, free radicals, and neurodegeneration

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

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

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

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

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Transglutaminase activity is related to CAG repeat length in patients with Huntington's disease

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Transcriptional dysregulation in Huntington's disease

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Epidermal immunization by a needle-free powder delivery technology: Immunogenicity of influenza vaccine and protection in mice

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

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

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

A quick and simple method for the quantitation of lactate dehydrogenase release in measurements of cellular cytotoxicity and tumor necrosis factor (TNF) activity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Effect of exogenous and endogenous antioxidants on 3-nitropionic acid-induced in vivo oxidative stress and striatal lesions: insights into Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

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

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Mitochondrial defect in Huntington's disease caudate nucleus

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Minocycline is protective in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Inefficient degradation of truncated polyglutamine proteins by the proteasome

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

On the active site thiol of gamma-glutamylcysteine synthetase: relationships to catalysis, inhibition, and regulation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Therapeutic opportunities in polyglutamine disease

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

N(epsilon)-(gamma-L-glutamyl)-L-lysine (GGEL) is increased in cerebrospinal fluid of patients with Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Mechanism for the inhibition of transglutaminase 2 by cystamine

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Cystamine inhibits caspase activity. Implications for the treatment of polyglutamine disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Tissue transglutaminase: a possible role in neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Impaired mitochondrial function results in increased tissue transglutaminase activity in situ

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Huntington's disease: a synaptopathy?

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Formation of γ-glutamyl-ε-lysine bridges between membrane proteins by a Ca2+-regulated enzyme in intact erythrocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

'Tissue' transglutaminase ablation reduces neuronal death and prolongs survival in a mouse model of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Treatment of YAC128 mice and their wild-type littermates with cystamine does not lead to its accumulation in plasma or brain: implications for the treatment of Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Riluzole therapy in Huntington's disease (HD).

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Striatal cells from mutant huntingtin knock-in mice are selectively vulnerable to mitochondrial complex II inhibitor-induced cell death through a non-apoptotic pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

The role of mitochondrial dysfunction and neuronal nitric oxide in animal models of neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Intracellular heterogeneity in mitochondrial membrane potentials revealed by a J-aggregate-forming lipophilic cation JC-1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Cystamine treatment is neuroprotective in the YAC128 mouse model of Huntington disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

Cell-permeable peptide antioxidants targeted to inner mitochondrial membrane inhibit mitochondrial swelling, oxidative cell death, and reperfusion injury

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1460-9568.2006.04686.X

10.1111/J.1460-9568.2006.04686.X

21 January 2018

Identifiers

DOI

1 reference

19 September 2017

1 reference