Mitochondrial calcium uptake capacity as a therapeutic target in the R6/2 mouse model of Huntington's disease (Q34046887)

30 July 2017

title

Mitochondrial calcium uptake capacity as a therapeutic target in the R6/2 mouse model of Huntington's disease (English)

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30 July 2017

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Giselle M Perry

1

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30 July 2017

Sara Tallaksen-Greene

2

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30 July 2017

Ashish Kumar

3

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30 July 2017

Mary Y Heng

4

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30 July 2017

Andrew Kneynsberg

5

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30 July 2017

Thomas van Groen

6

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30 July 2017

Peter J Detloff

7

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30 July 2017

Roger L Albin

8

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30 July 2017

Mathieu Lesort

9

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30 July 2017

publication date

17 June 2010

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30 July 2017

published in

Human Molecular Genetics

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30 July 2017

volume

19

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30 July 2017

issue

17

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30 July 2017

page(s)

3354-3371

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Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models

30 July 2017

cites work

1 reference

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5 July 2018

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Cyclophilin D deficiency attenuates mitochondrial and neuronal perturbation and ameliorates learning and memory in Alzheimer's disease

5 July 2018

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5 July 2018

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Impaired regulation of brain mitochondria by extramitochondrial Ca2+ in transgenic Huntington disease rats

5 July 2018

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5 July 2018

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

5 July 2018

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5 July 2018

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Cyclophilin D inactivation protects axons in experimental autoimmune encephalomyelitis, an animal model of multiple sclerosis

5 July 2018

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Voltage-dependent anion channels are dispensable for mitochondrial-dependent cell death

5 July 2018

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

5 July 2018

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

5 July 2018

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Cyclophilin D is a component of mitochondrial permeability transition and mediates neuronal cell death after focal cerebral ischemia

5 July 2018

1 reference

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Age-dependent changes in the calcium sensitivity of striatal mitochondria in mouse models of Huntington's Disease

5 July 2018

1 reference

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Loss of cyclophilin D reveals a critical role for mitochondrial permeability transition in cell death

5 July 2018

1 reference

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Cyclophilin D-dependent mitochondrial permeability transition regulates some necrotic but not apoptotic cell death

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Properties of the permeability transition pore in mitochondria devoid of Cyclophilin D.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Huntington's disease genetics

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Standardization and statistical approaches to therapeutic trials in the R6/2 mouse

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Evidence for more widespread cerebral pathology in early HD: an MRI-based morphometric analysis

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Mitochondrial targeted cyclophilin D protects cells from cell death by peptidyl prolyl isomerization

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Striatal volume loss in HD as measured by MRI and the influence of CAG repeat

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Dramatic mutation instability in HD mouse striatum: does polyglutamine load contribute to cell-specific vulnerability in Huntington's disease?

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

The mitochondrial permeability transition pore

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Trinucleotide instability: a repeating theme in human inherited disorders

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Instability of CAG repeats in Huntington's disease: relation to parental transmission and age of onset

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Somatic and gonadal mosaicism of the Huntington disease gene CAG repeat in brain and sperm.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Huntington's disease, energy, and excitotoxicity

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

NMDA receptor losses in putamen from patients with Huntington's disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Alternative excitotoxic hypotheses

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Juvenile Huntington chorea: Clinical, ultrastructural, and biochemical studies

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

In vivo evidence for NMDA receptor-mediated excitotoxicity in a murine genetic model of Huntington disease.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice.

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Increased glutathione levels in cortical and striatal mitochondria of the R6/2 Huntington's disease mouse model

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Excitotoxic injury to mitochondria isolated from cultured neurons.

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Verification of somatic CAG repeat expansion by pre-PCR fractionation

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

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

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Brain Mitochondria Are Primed by Moderate Ca2+ Rise upon Hypoxia/Reoxygenation for Functional Breakdown and Morphological Disintegration

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Regional and progressive thinning of the cortical ribbon in Huntington's disease

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Huntingtin aggregate-associated axonal degeneration is an early pathological event in Huntington's disease mice.

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy.

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Biochemical abnormalities and excitotoxicity in Huntington's disease brain.

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Reduced penetrance of the Huntington's disease mutation

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Proton magnetic resonance spectroscopy in Huntington's disease: evidence in favour of the glutamate excitotoxic theory

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Excitotoxin lesions in primates as a model for Huntington's disease: histopathologic and neurochemical characterization

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2916705

Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

28 October 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/20558522

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Glutamate induces the production of reactive oxygen species in cultured forebrain neurons following NMDA receptor activation

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/20558522

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1093/HMG/DDQ247

1 reference

30 July 2017

1 reference

30 July 2017

1 reference