α-Synuclein accumulates in huntingtin inclusions but forms independent filaments and its deficiency attenuates early phenotype in a mouse model of Huntington's disease. (Q45305036)

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English	α-Synuclein accumulates in huntingtin inclusions but forms independent filaments and its deficiency attenuates early phenotype in a mouse model of Huntington's disease.	scientific article

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15 January 2020

α-Synuclein accumulates in huntingtin inclusions but forms independent filaments and its deficiency attenuates early phenotype in a mouse model of Huntington's disease (English)

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15 January 2020

Huntington's disease

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based on heuristic

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15 January 2020

Cristina Tomás-Zapico

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15 January 2020

María Teresa Miras Portugal

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15 January 2020

Miguel Diaz-Hernandez

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15 January 2020

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Isidre Ferrer

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15 January 2020

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María Díez-Zaera

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15 January 2020

Pilar Gómez-Ramos

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15 January 2020

María A Morán

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15 January 2020

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1 November 2011

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15 January 2020

Human Molecular Genetics

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15 January 2020

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15 January 2020

3

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15 January 2020

495-510

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15 January 2020

Trinucleotide Repeat Disorders

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Protein aggregation and neurodegenerative disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Progressive disruption of cellular protein folding in models of polyglutamine diseases.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Alpha-synuclein cooperates with CSPalpha in preventing neurodegeneration

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Alpha-synuclein promotes SNARE-complex assembly in vivo and in vitro

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Alpha-synuclein and the Lewy body disorders

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

alpha-Synuclein in filamentous inclusions of Lewy bodies from Parkinson's disease and dementia with lewy bodies

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Accelerated in vitro fibril formation by a mutant alpha-synuclein linked to early-onset Parkinson disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Both familial Parkinson's disease mutations accelerate alpha-synuclein aggregation

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Mutation in the alpha-synuclein gene identified in families with Parkinson's disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Ala30Pro mutation in the gene encoding alpha-synuclein in Parkinson's disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

The new mutation, E46K, of alpha-synuclein causes Parkinson and Lewy body dementia

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

alpha-Synuclein locus triplication causes Parkinson's disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Alpha-synuclein immunoreactivity of huntingtin polyglutamine aggregates in striatum and cortex of Huntington's disease patients and transgenic mouse models

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

α-Synuclein impairs macroautophagy: implications for Parkinson's disease.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Biochemical, ultrastructural, and reversibility studies on huntingtin filaments isolated from mouse and human brain.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Mice lacking alpha-synuclein display functional deficits in the nigrostriatal dopamine system

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Fibrils formed in vitro from alpha-synuclein and two mutant forms linked to Parkinson's disease are typical amyloid

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Opinion: What is the role of protein aggregation in neurodegeneration?

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Accumulation of ubiquitin conjugates in a polyglutamine disease model occurs without global ubiquitin/proteasome system impairment

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Delaying the onset of Huntington's in mice

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Molecular and behavioral analysis of the R6/1 Huntington's disease transgenic mouse

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Effects of partial suppression of parkin on huntingtin mutant R6/1 mice.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

The detection and measurement of locomotor deficits in a transgenic mouse model of Huntington's disease are task- and protocol-dependent: influence of non-motor factors on locomotor function

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Specificity in intracellular protein aggregation and inclusion body formation

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Autophagy: links with the proteasome

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Impairment of the ubiquitin-proteasome system by protein aggregation

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Inhibition of 26S proteasome activity by huntingtin filaments but not inclusion bodies isolated from mouse and human brain

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Acute polyglutamine expression in inducible mouse model unravels ubiquitin/proteasome system impairment and permanent recovery attributable to aggregate formation.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Gaucher disease glucocerebrosidase and α-synuclein form a bidirectional pathogenic loop in synucleinopathies

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

"Emerging Alzheimer's disease therapies: focusing on the future".

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Concurrence of alpha-synuclein and tau brain pathology in the Contursi kindred

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Initiation and Synergistic Fibrillization of Tau and Alpha-Synuclein

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

beta-amyloid peptides enhance alpha-synuclein accumulation and neuronal deficits in a transgenic mouse model linking Alzheimer's disease and Parkinson's disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Cross-seeding fibrillation of Q/N-rich proteins offers new pathomechanism of polyglutamine diseases

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Dynamic interactions of Sup35p and PrP prion protein domains modulate aggregate nucleation and seeding

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Huntington disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Deletion of the alpha-synuclein locus in a subpopulation of C57BL/6J inbred mice

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Decreased nuclear beta-catenin, tau hyperphosphorylation and neurodegeneration in GSK-3beta conditional transgenic mice

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDR507

21 January 2018

Identifiers

10.1093/HMG/DDR507

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22045698%20AND%20SRC:MED&resulttype=core&format=json

15 January 2020

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:22045698%20AND%20SRC:MED&resulttype=core&format=json

15 January 2020

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