(Q35747131)

English

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

scientific article published on 13 October 2011

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Europe PubMed Central

PubMed publication ID

10 August 2017

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

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Europe PubMed Central

PubMed publication ID

10 August 2017

Huntington's disease

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Brett Dufour

5

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3

object named as

Maria Manczak

1 reference

Europe PubMed Central

PubMed publication ID

10 August 2017

author name string

Ulziibat P Shirendeb

1

1 reference

Europe PubMed Central

PubMed publication ID

10 August 2017

Marcus J Calkins

2

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Europe PubMed Central

PubMed publication ID

10 August 2017

Vishwanath Anekonda

4

1 reference

Europe PubMed Central

PubMed publication ID

10 August 2017

Jodi L McBride

6

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Europe PubMed Central

PubMed publication ID

10 August 2017

Peizhong Mao

7

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Europe PubMed Central

PubMed publication ID

10 August 2017

P Hemachandra Reddy

8

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Europe PubMed Central

PubMed publication ID

10 August 2017

publication date

13 October 2011

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Europe PubMed Central

PubMed publication ID

10 August 2017

Human Molecular Genetics

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Europe PubMed Central

PubMed publication ID

10 August 2017

21

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Europe PubMed Central

PubMed publication ID

10 August 2017

2

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Europe PubMed Central

PubMed publication ID

10 August 2017

406-420

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Europe PubMed Central

PubMed publication ID

10 August 2017

describes a project that uses

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Europe PubMed Central

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC3276281/fullTextXML

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Impaired mitochondrial biogenesis, defective axonal transport of mitochondria, abnormal mitochondrial dynamics and synaptic degeneration in a mouse model of Alzheimer's disease

1 reference

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Peroxisome-proliferator-activated receptor gamma coactivator 1 α contributes to dysmyelination in experimental models of Huntington's disease

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Assessment of newly synthesized mitochondrial DNA using BrdU labeling in primary neurons from Alzheimer's disease mice: Implications for impaired mitochondrial biogenesis and synaptic damage

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Mutant huntingtin causes metabolic imbalance by disruption of hypothalamic neurocircuits

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Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity

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Early changes in the hypothalamic region in prodromal Huntington disease revealed by MRI analysis

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Mitochondria-targeted antioxidants protect against amyloid-beta toxicity in Alzheimer's disease neurons

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Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression

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Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice

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Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease

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Mitochondria: a therapeutic target in neurodegeneration

1 reference

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12 July 2018

The dynamin-related GTPase Drp1 is required for embryonic and brain development in mice

1 reference

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12 July 2018

Mitochondrial fission factor Drp1 is essential for embryonic development and synapse formation in mice

1 reference

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

1 reference

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

1 reference

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Mitochondrial structural and functional dynamics in Huntington's disease

1 reference

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Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models

1 reference

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Effects of overexpression of huntingtin proteins on mitochondrial integrity

1 reference

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Progressive synaptic pathology of motor cortical neurons in a BAC transgenic mouse model of Huntington's disease

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Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice

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

1 reference

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Huntington disease models and human neuropathology: similarities and differences

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Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity

1 reference

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Hypothesis: Huntingtin may function in membrane association and vesicular trafficking

1 reference

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

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1 reference

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Huntingtin associates with acidic phospholipids at the plasma membrane

1 reference

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

1 reference

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Rate and correlates of weight change in Huntington's disease

1 reference

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

1 reference

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

1 reference

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Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

1 reference

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

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Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse

1 reference

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

1 reference

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

1 reference

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

1 reference

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

1 reference

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Neuronal loss in the hippocampus in Huntington's disease: a comparison with HIV infection

1 reference

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

1 reference

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

1 reference

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Huntington's disease in children. Neuropathologic study of four cases

1 reference

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Neuropathological classification of Huntington's disease

1 reference

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Weight loss in Huntington disease increases with higher CAG repeat number

1 reference

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Hypothalamic involvement in Huntington's disease: an in vivo PET study

1 reference

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Wild-type huntingtin participates in protein trafficking between the Golgi and the extracellular space

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3276281

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

1 reference

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25 September 2018

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

1 reference

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The use of real-time PCR analysis in a gene expression study of Alzheimer's disease post-mortem brains

1 reference

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25 September 2018

Huntington's disease: clinical correlates of disability and progression

1 reference

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25 September 2018

Progression of symptoms in the early and middle stages of Huntington disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3276281

25 September 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3276281

25 September 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3276281

25 September 2018

Regional specificity of brain atrophy in Huntington's disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3276281

25 September 2018

Identifiers

10.1093/HMG/DDR475

1 reference

Europe PubMed Central

PubMed publication ID

10 August 2017

PMC publication ID

1 reference

Europe PubMed Central

PubMed publication ID

10 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

10 August 2017

ResearchGate publication ID

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