(Q83728053)

English

Huntington's Disease

scientific article published on 01 June 2011

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scholarly article

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Europe PubMed Central

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26 January 2020

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Europe PubMed Central

Huntington's Disease (English)

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Europe PubMed Central

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26 January 2020

Huntington's disease

1 reference

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author name string

Steven Finkbeiner

1

1 reference

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26 January 2020

publication date

1 June 2011

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Europe PubMed Central

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26 January 2020

Cold Spring Harbor Perspectives in Biology

1 reference

Europe PubMed Central

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26 January 2020

3

1 reference

Europe PubMed Central

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26 January 2020

6

1 reference

Europe PubMed Central

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26 January 2020

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Overexpression of yeast hsp104 reduces polyglutamine aggregation and prolongs survival of a transgenic mouse model of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Functional amyloid formation within mammalian tissue

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Proteasome impairment does not contribute to pathogenesis in R6/2 Huntington's disease mice: exclusion of proteasome activator REGgamma as a therapeutic target

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Evidence for a recruitment and sequestration mechanism in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Polyglutamine expansion, protein aggregation, proteasome activity, and neural survival.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Genetic suppression of polyglutamine toxicity in Drosophila

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Are Huntington's and polyglutamine-based ataxias proteasome storage diseases?

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aberrant excitatory neuronal activity and compensatory remodeling of inhibitory hippocampal circuits in mouse models of Alzheimer's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Can autophagy protect against neurodegeneration caused by aggregate-prone proteins?

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A modified beta-amyloid hypothesis: intraneuronal accumulation of the beta-amyloid peptide--the first step of a fatal cascade

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Intracellular inclusions, pathological markers in diseases caused by expanded polyglutamine tracts?

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and alpha-synuclein.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Sequestosome 1/p62 shuttles polyubiquitinated tau for proteasomal degradation

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Pathogenic and Non-pathogenic Polyglutamine Tracts Have Similar Structural Properties: Towards a Length-dependent Toxicity Gradient

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Molecular analysis of juvenile Huntington disease: the major influence on (CAG)n repeat length is the sex of the affected parent.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Differential expression of normal and mutant Huntington's disease gene alleles

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Twenty years of the Alzheimer's disease amyloid hypothesis: a genetic perspective

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Involvement of macroautophagy in the dissolution of neuronal inclusions.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aggresomes: a cellular response to misfolded proteins

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Protein disaggregation mediated by heat-shock protein Hsp104.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Autophagy, proteasomes, lipofuscin, and oxidative stress in the aging brain.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

RNA toxicity is a component of ataxin-3 degeneration in Drosophila

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Immunocytochemical studies of substance P and leucine-enkephalin in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Membrane filter assay for detection of amyloid-like polyglutamine-containing protein aggregates.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Autophagy and aging: the importance of maintaining "clean" cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Intranuclear inclusions and the ubiquitin-proteasome pathway: digestion of a red herring?

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Regulation and role of autophagy in mammalian cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurodegeneration: aging and dementia. Etiopathogenic role of electron transport disorders. Therapeutic possibilities

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

PolyQ disease: too many Qs, too much function?

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Common structure and toxic function of amyloid oligomers implies a common mechanism of pathogenesis

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Small molecules enhance autophagy and reduce toxicity in Huntington's disease models.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A structure-based analysis of huntingtin mutant polyglutamine aggregation and toxicity: evidence for a compact beta-sheet structure.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Common structure of soluble amyloid oligomers implies common mechanism of pathogenesis

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Emerging role for autophagy in the removal of aggresomes in Schwann cells.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Polyglutamine protein aggregates are dynamic

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The ubiquitin-proteasome pathway and proteasome inhibitors

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Sodium dodecyl sulfate-insoluble oligomers are involved in polyglutamine degeneration.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aggregation of truncated GST-HD exon 1 fusion proteins containing normal range and expanded glutamine repeats

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

PET study of the pre- and post-synaptic dopaminergic markers for the neurodegenerative process in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Molecular chaperones: clasping the prize

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Proteasomes and proteasome inhibition in the central nervous system

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expanded polyglutamine stretches form an 'aggresome'.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Amyloid-like features of polyglutamine aggregates and their assembly kinetics

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The aggravating role of the ubiquitin-proteasome system in neurodegeneration

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neurodegenerative disease: neuron protection agency

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Absence of disease phenotype and intergenerational stability of the CAG repeat in transgenic mice expressing the human Huntington disease transcript

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aging as an event of proteostasis collapse

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Increased expression of p62 in expanded polyglutamine-expressing cells and its association with polyglutamine inclusions.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

ApoE isoform-specific effects on LTP: blockade by oligomeric amyloid-beta1-42.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Caretaker or undertaker? The role of the proteasome in aging

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Hsp70 and aging

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Impairment of the ubiquitin-proteasome system by protein aggregation

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The ubiquitin-related BAG-1 provides a link between the molecular chaperones Hsc70/Hsp70 and the proteasome

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Network-level neuroplasticity in cortico-basal ganglia pathways

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Regional cortical thinning in preclinical Huntington disease and its relationship to cognition

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A one-hit model of cell death in inherited neuronal degenerations

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Dendritic cell aggresome-like induced structures are dedicated areas for ubiquitination and storage of newly synthesized defective proteins

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The autophagy-lysosomal degradation pathway: role in neurodegenerative disease and therapy.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntingtin aggregation monitored by dynamic light scattering

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neuropathological classification of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

SCA7 knockin mice model human SCA7 and reveal gradual accumulation of mutant ataxin-7 in neurons and abnormalities in short-term plasticity.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inhibition of polyglutamine aggregate cytotoxicity by a structure-based elongation inhibitor

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The deacetylase HDAC6 regulates aggresome formation and cell viability in response to misfolded protein stress

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Nuclear inclusions in glutamine repeat disorders: are they pernicious, coincidental, or beneficial?

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The IGF-1/Akt pathway is neuroprotective in Huntington's disease and involves Huntingtin phosphorylation by Akt.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Oligomerization of expanded-polyglutamine domain fluorescent fusion proteins in cultured mammalian cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Chaperone-mediated autophagy in aging and disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

History of genetic disease: the molecular genetics of Huntington disease - a history

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Microtubule disruption inhibits autophagosome-lysosome fusion: implications for studying the roles of aggresomes in polyglutamine diseases

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntington aggregates may not predict neuronal death in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Reduction in enkephalin and substance P messenger RNA in the striatum of early grade Huntington's disease: a detailed cellular in situ hybridization study

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Recent advances in understanding the pathogenesis of polyglutamine diseases: involvement of molecular chaperones and ubiquitin-proteasome pathway.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The chaperonin TRiC controls polyglutamine aggregation and toxicity through subunit-specific interactions

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Modulation of neurodegeneration by molecular chaperones

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Anticipation and instability of IT-15 (CAG)n repeats in parent-offspring pairs with Huntington disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Presymptomatic compensation in Parkinson's disease is not dopamine-mediated

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Single neuron ubiquitin-proteasome dynamics accompanying inclusion body formation in huntington disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Hsp104, Hsp70, and Hsp40: a novel chaperone system that rescues previously aggregated proteins

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Preferential loss of preproenkephalin versus preprotachykinin neurons from the striatum of Huntington's disease patients

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mechanism of neurodegenerative disease: role of the ubiquitin proteasome system

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aging, energy, and oxidative stress in neurodegenerative diseases.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Pharmacological promotion of inclusion formation: a therapeutic approach for Huntington's and Parkinson's diseases

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A new hypothesis of neurodegenerative diseases: the deleterious network hypothesis

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Trinucleotide repeat length instability and age of onset in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Protein conformation and disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

CAG repeat number governs the development rate of pathology in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Yeast genes that enhance the toxicity of a mutant huntingtin fragment or alpha-synuclein

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Solution structure of polyglutamine tracts in GST-polyglutamine fusion proteins

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inefficient degradation of truncated polyglutamine proteins by the proteasome

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Hsp70 and Hsp40 attenuate formation of spherical and annular polyglutamine oligomers by partitioning monomer

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Clinical and neuropathologic assessment of severity in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Autophagy-mediated clearance of huntingtin aggregates triggered by the insulin-signaling pathway

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Genome-wide RNA interference screen identifies previously undescribed regulators of polyglutamine aggregation

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Unraveling a role for dopamine in Huntington's disease: the dual role of reactive oxygen species and D2 receptor stimulation

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Dopamine receptor gene expression by enkephalin neurons in rat forebrain.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Evidence of cortical metabolic dysfunction in early Huntington's disease by single-photon-emission computed tomography

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gametic but not somatic instability of CAG repeat length in Huntington's disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The ubiquitin-proteasome pathway in Huntington's disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Pathway for degradation of peptides generated by proteasomes: a key role for thimet oligopeptidase and other metallopeptidases

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Permeabilization of lipid bilayers is a common conformation-dependent activity of soluble amyloid oligomers in protein misfolding diseases

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

p62 Is a common component of cytoplasmic inclusions in protein aggregation diseases

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Autophagy regulates the processing of amino terminal huntingtin fragments

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntington's disease: seeing the pathogenic process through a genetic lens

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Trinucleotide repeat length and clinical progression in Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The ubiquitin proteasome system in neurodegenerative diseases: sometimes the chicken, sometimes the egg.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Proteasome degrades soluble expanded polyglutamine completely and efficiently

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntingtin forms toxic NH2-terminal fragment complexes that are promoted by the age-dependent decrease in proteasome activity.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Somatic mosaicism in sperm is associated with intergenerational (CAG)n changes in Huntington disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Juvenile onset Huntington's disease--clinical and research perspectives.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Characterizing the conformational ensemble of monomeric polyglutamine.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Automated microscope system for determining factors that predict neuronal fate.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

CREB-binding protein sequestration by expanded polyglutamine

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

SCA1-like disease in mice expressing wild-type ataxin-1 with a serine to aspartic acid replacement at residue 776.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Prevention of polyglutamine oligomerization and neurodegeneration by the peptide inhibitor QBP1 in Drosophila

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Autophagy-mediated clearance of aggresomes is not a universal phenomenon

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A toxic monomeric conformer of the polyglutamine protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Pathological synaptic plasticity in the striatum: implications for Parkinson's disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Autophagy, cytoplasm-to-vacuole targeting pathway, and pexophagy in yeast and mammalian cells.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Secondary Structure of Huntingtin Amino-Terminal Region

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Extended polyglutamine tracts cause aggregation and structural perturbation of an adjacent beta barrel protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Hsp70 and hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Detection of Huntington's disease decades before diagnosis: the Predict-HD study

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Duplication of Atxn1l suppresses SCA1 neuropathology by decreasing incorporation of polyglutamine-expanded ataxin-1 into native complexes.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Disease-modifying pathways in neurodegeneration.

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/21441583

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1101/CSHPERSPECT.A007476

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21441583%20AND%20SRC:MED&resulttype=core&format=json

26 January 2020

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21441583%20AND%20SRC:MED&resulttype=core&format=json

26 January 2020

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:21441583%20AND%20SRC:MED&resulttype=core&format=json

26 January 2020

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