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Lista de obras de Gillian Bates

A brain-permeable small molecule reduces neuronal cholesterol by inhibiting activity of sirtuin 2 deacetylase

artículo científico publicado en 2011

A candidate for the cystic fibrosis locus isolated by selection for methylation-free islands

artículo científico publicado en 1987

A common gene expression signature in Huntington's disease patient brain regions

artículo científico publicado en 2014

A human single-chain Fv intrabody preferentially targets amino-terminal Huntingtin's fragments in striatal models of Huntington's disease

artículo científico publicado en 2005

A large number of protein expression changes occur early in life and precede phenotype onset in a mouse model for huntington disease

artículo científico publicado en 2008

A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis

artículo científico publicado en 1998

A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease

artículo científico publicado en 2008

A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo

artículo científico publicado en 2005

Aberrant processing of the Fugu HD (FrHD) mRNA in mouse cells and in transgenic mice

artículo científico publicado en 1997

Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease

artículo científico publicado en 2013

Aberrantly spliced HTT, a new player in Huntington's disease pathogenesis

artículo científico publicado en 2013

Abnormal phosphorylation of synapsin I predicts a neuronal transmission impairment in the R6/2 Huntington's disease transgenic mice

artículo científico publicado en 2002

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

artículo científico publicado en 1997

Alterations in the mouse and human proteome caused by Huntington's disease

artículo científico publicado en 2002

Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene

scientific article published on May 1998

Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease

artículo científico publicado en 2011

Amyloid-like inclusions in Huntington's disease.

artículo científico publicado en 2000

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

artículo científico publicado en 1998

Arfaptin 2 regulates the aggregation of mutant huntingtin protein

artículo científico publicado en 2002

BIOMEDICINE: One Misfolded Protein Allows Others to Sneak By.

artículo científico publicado en 2006

Biochemical and genetic exclusion of calmodulin as the site of the basic defect in cystic fibrosis.

artículo científico publicado en 1987

Biomarkers for neurodegenerative diseases

artículo científico publicado en 2005

Brain neurotransmitter deficits in mice transgenic for the Huntington's disease mutation

artículo científico publicado en 1999

CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease

artículo científico publicado en 2010

Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 knock-in mouse model of Huntington's disease

artículo científico publicado en 2012

Changes in GAD67 mRNA expression evidenced by in situ hybridization in the brain of R6/2 transgenic mice.

artículo científico publicado en 2003

Characterisation of immune cell function in fragment and full-length Huntington's disease mouse models

artículo científico publicado en 2014

Characterization of Gastric Mucosa Biopsies Reveals Alterations in Huntington's Disease

artículo científico publicado en 2015

Cloning of the alpha-adducin gene from the Huntington's disease candidate region of chromosome 4 by exon amplification

artículo científico publicado el 1 de noviembre de 1992

Complex alteration of NMDA receptors in transgenic Huntington's disease mouse brain: analysis of mRNA and protein expression, plasma membrane association, interacting proteins, and phosphorylation

artículo científico publicado en 2003

Complex patterns of linkage disequilibrium in the Huntington disease region ⬇️

artículo científico publicado el 1 de octubre de 1991

Contesting the dogma of an age-related heat shock response impairment: implications for cardiac-specific age-related disorders

artículo científico publicado en 2014

Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease

artículo científico publicado en 2005

Correction: SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice

artículo científico publicado en 2016

Correlations of Behavioral Deficits with Brain Pathology Assessed through Longitudinal MRI and Histopathology in the HdhQ150/Q150 Mouse Model of Huntington's Disease

artículo científico publicado en 2017

Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/1 mouse model of Huntington's disease

artículo científico publicado en 2013

Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/2 mouse model of HD

artículo científico publicado en 2013

Correlative light and electron microscopy suggests that mutant huntingtin dysregulates the endolysosomal pathway in presymptomatic Huntington's disease

artículo científico publicado en 2021

DNA instability in postmitotic neurons

artículo científico publicado en 2008

Defined physical limits of the Huntington disease gene candidate region ⬇️

artículo científico publicado el 1 de julio de 1991

Depletion of rabphilin 3A in a transgenic mouse model (R6/1) of Huntington's disease, a possible culprit in synaptic dysfunction

artículo científico publicado en 2005

Detection of polyglutamine aggregation in mouse models

artículo científico publicado en 1999

Disruption to schizophrenia-associated gene Fez1 in the hippocampus of HDAC11 knockout mice

artículo científico publicado en 2017

Downregulation of cannabinoid receptor 1 from neuropeptide Y interneurons in the basal ganglia of patients with Huntington's disease and mouse models.

artículo científico publicado en 2012

Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies

artículo científico publicado en 2013

Dysfunction of the CNS-heart axis in mouse models of Huntington's disease

artículo científico publicado en 2014

Dysfunction of the cholesterol biosynthetic pathway in Huntington's disease.

artículo científico publicado en 2005

Early and transient alteration of adenosine A2A receptor signaling in a mouse model of Huntington disease

artículo científico publicado en 2006

Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice

scientific journal article

Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington's Disease

artículo científico publicado en 2016

Environmental enrichment slows disease progression in R6/2 Huntington's disease mice

artículo científico publicado en 2002

Evaluation of the benzothiazole aggregation inhibitors riluzole and PGL-135 as therapeutics for Huntington's disease

artículo científico publicado en 2005

Exendin-4 improves glycemic control, ameliorates brain and pancreatic pathologies, and extends survival in a mouse model of Huntington's disease

artículo científico publicado en 2009

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

artículo científico publicado en 1996

Exon trapping and sequence-based methods of gene finding in transcript mapping of human 4p 16.3

article

Expanded glutamines and neurodegeneration--a gain of insight

artículo científico publicado en 1996

Experimental therapeutics in Huntington's disease: are models useful for therapeutic trials?

artículo científico publicado en 2003

Expression of mutant exon 1 huntingtin fragments in human neural stem cells and neurons causes inclusion formation and mitochondrial dysfunction

scientific article published on 23 April 2020

Extensive Expression Analysis of Htt Transcripts in Brain Regions from the zQ175 HD Mouse Model Using a QuantiGene Multiplex Assay

scientific article published on 06 November 2019

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

artículo científico publicado en 1997

Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease

artículo científico publicado en 2009

Fragments of HdhQ150 mutant huntingtin form a soluble oligomer pool that declines with aggregate deposition upon aging

artículo científico publicado en 2012

Frequency of nuclear mutant huntingtin inclusion formation in neurons and glia is cell-type-specific.

artículo científico publicado en 2016

Gastrointestinal dysfunction contributes to weight loss in Huntington's disease mice

artículo científico publicado en 2011

Generation of high-density DNA markers from yeast artificial chromosome DNA by single unique primer-polymerase chain reaction

artículo científico publicado en 1993

Genetic deletion of S6k1 does not rescue the phenotypic deficits observed in the R6/2 mouse model of Huntington's disease

scientific article published on 06 November 2019

Genetic knock-down of HDAC3 does not modify disease-related phenotypes in a mouse model of Huntington's disease

artículo científico publicado en 2012

Genetic knock-down of HDAC7 does not ameliorate disease pathogenesis in the R6/2 mouse model of Huntington's disease

artículo científico publicado en 2009

Global changes to the ubiquitin system in Huntington's disease.

artículo científico publicado en 2007

HDAC4 does not act as a protein deacetylase in the postnatal murine brain in vivo

artículo científico publicado en 2013

HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration

artículo científico publicado en 2013

HDAC4-myogenin axis as an important marker of HD-related skeletal muscle atrophy

artículo científico publicado en 2015

HSF1-dependent and -independent regulation of the mammalian in vivo heat shock response and its impairment in Huntington's disease mouse models

artículo científico publicado en 2017

HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation

artículo científico publicado en 2014

Hdac6 knock-out increases tubulin acetylation but does not modify disease progression in the R6/2 mouse model of Huntington's disease

artículo científico publicado en 2011

Histone deacetylase inhibitors as therapeutics for polyglutamine disorders

artículo científico publicado en 2006

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

artículo científico publicado en 2005

Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation

artículo científico publicado en 2007

Hunting in the calm before the storm

artículo científico publicado en 2000

Huntingtin

Huntingtin aggregation and toxicity in Huntington's disease ⬇️

artículo científico publicado el 10 de mayo de 2003

Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series

artículo científico publicado en 2004

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

artículo científico publicado en 1997

Huntington disease

Huntington's disease. Exploiting expression

artículo científico publicado en 2001

Huntington's disease. In reverse gear

artículo científico publicado en 2000

IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome.

artículo científico publicado en 2009

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

artículo científico publicado en 2010

Identification of an HD patient with a (CAG)180 repeat expansion and the propagation of highly expanded CAG repeats in lambda phage

artículo científico publicado en 1997

Impaired glutamate uptake in the R6 Huntington's disease transgenic mice.

artículo científico publicado en 2001

Implantation of undifferentiated and pre-differentiated human neural stem cells in the R6/2 transgenic mouse model of Huntington's disease

artículo científico publicado en 2012

In Vivo Profiling Reveals a Competent Heat Shock Response in Adult Neurons: Implications for Neurodegenerative Disorders

artículo científico publicado en 2015

In vivo neutralization of the protagonist role of macrophages during the chronic inflammatory stage of Huntington's disease

scientific article published in Scientific Reports

Inclusion formation in Huntington's disease R6/2 mouse muscle cultures

artículo científico publicado en 2003

Increased metabolism in the R6/2 mouse model of Huntington's disease.

artículo científico publicado en 2007

Inhibition of polyglutamine aggregation in R6/2 HD brain slices-complex dose-response profiles

artículo científico publicado en 2001

Inhibition of tumour necrosis factor alpha in the R6/2 mouse model of Huntington’s disease by etanercept treatment

article

Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation.

artículo científico publicado en 1997

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

artículo científico publicado en 1998

Isolation of YAC Ends by Plasmid Rescue

artículo científico publicado en 1996

Live-cell super-resolution microscopy reveals a primary role for diffusion in polyglutamine-driven aggresome assembly

artículo científico publicado en 2018

Loss of cortical and thalamic neuronal tenascin-C expression in a transgenic mouse expressing exon 1 of the human Huntington disease gene

artículo científico publicado en 2001

Meso scale discovery-based assays for the detection of aggregated huntingtin

artículo científico publicado en 2019

Metabolic characterization of the R6/2 transgenic mouse model of Huntington's disease by high-resolution MAS 1H NMR spectroscopy

artículo científico publicado en 2006

Minocycline and doxycycline are not beneficial in a model of Huntington's disease

artículo científico publicado en 2003

Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse

artículo científico publicado en 2000

Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse.

artículo científico publicado en 2000

Monitoring aggregate formation in organotypic slice cultures from transgenic mice

artículo científico publicado en 2004

Mouse Models of Huntington's Disease

artículo científico publicado en 2018

Mouse Models of Triplet Repeat Diseases

artículo científico publicado en 2006

Mouse models of triplet repeat diseases

artículo científico publicado en 2004

Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression

artículo científico publicado en 2012

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

artículo científico publicado en 2007

Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington's disease mice

artículo científico publicado en 2017

New DNA markers in the Huntington's disease gene candidate region ⬇️

artículo científico publicado el 1 de septiembre de 1991

Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease

artículo científico publicado en 2000

Novel isoforms of heat shock transcription factor 1, HSF1γα and HSF1γβ, regulate chaperone protein gene transcription

artículo científico publicado en 2014

Optimisation of region-specific reference gene selection and relative gene expression analysis methods for pre-clinical trials of Huntington's disease

artículo científico publicado en 2008

Oral administration of the pimelic diphenylamide HDAC inhibitor HDACi 4b is unsuitable for chronic inhibition of HDAC activity in the CNS in vivo

artículo científico publicado en 2012

Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length

artículo científico publicado en 2001

Phenotype onset in Huntington's disease knock-in mice is correlated with the incomplete splicing of the mutant huntingtin gene

scientific article published on 07 July 2019

Polyglutamine expansion of huntingtin impairs its nuclear export

artículo científico publicado en 2005

Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mouse model of Huntington's disease

artículo científico publicado en 2006

Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach

artículo científico publicado en 2004

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

artículo científico publicado en 2005

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

article published in 2006

Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease

artículo científico publicado en 2010

Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates

artículo científico publicado en 2007

RFLP for D4S12, an anonymous single copy genomic clone at 4pter-4q26 [HGM8 provisional no. D4S12]

artículo científico publicado en 1985

RFLP for pHM20 (D2S12), an anonymous DNA sequence localised to 2p23-2pter

artículo científico publicado en 1987

RNA Related Pathology in Huntington's Disease

artículo científico publicado en 2018

Radiation hybrid map spanning the Huntington disease gene region of chromosome 4.

artículo científico publicado en 1992

Reducing Igf-1r levels leads to paradoxical and sexually dimorphic effects in HD mice

artículo científico publicado en 2014

Reduction of GnRH and infertility in the R6/2 mouse model of Huntington's disease

artículo científico publicado en 2005

Regional localization of the gene coding for human brain nitric oxide synthase (NOS1) to 12q24.2-->24.31 by fluorescent in situ hybridization

artículo científico publicado en 1993

Regulatory mechanisms of incomplete huntingtin mRNA splicing

artículo científico publicado en 2018

SAHA decreases HDAC 2 and 4 levels in vivo and improves molecular phenotypes in the R6/2 mouse model of Huntington's disease

artículo científico publicado en 2011

SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice

artículo científico publicado en 2016

SIRT2 ablation has no effect on tubulin acetylation in brain, cholesterol biosynthesis or the progression of Huntington's disease phenotypes in vivo

artículo científico publicado en 2012

SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis

artículo científico publicado en 2010

SUMO-2 and PIAS1 modulate insoluble mutant huntingtin protein accumulation

artículo científico publicado en 2013

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

artículo científico publicado en 1999

Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease

artículo científico publicado en 2007

Sequence comparison of human and yeast telomeres identifies structurally distinct subtelomeric domains

artículo científico publicado en 1997

Silencing Srsf6 does not modulate incomplete splicing of the huntingtin gene in Huntington's disease models

artículo científico publicado en 2020

Stall in Canonical Autophagy-Lysosome Pathways Prompts Nucleophagy-Based Nuclear Breakdown in Neurodegeneration

artículo científico publicado en 2017

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

artículo científico publicado en 2003

Striatal transplantation in a transgenic mouse model of Huntington's disease

artículo científico publicado en 1998

Striking changes in anxiety in Huntington's disease transgenic mice

artículo científico publicado en 1998

Structure and expression of the Huntington's disease gene: evidence against simple inactivation due to an expanded CAG repeat

artículo científico publicado en 1994

Subcellular Localization And Formation Of Huntingtin Aggregates Correlates With Symptom Onset And Progression In A Huntington'S Disease Model

scientific article published on 03 August 2020

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

artículo científico publicado en 2003

Suppression of protein aggregation by chaperone modification of high molecular weight complexes

artículo científico publicado en 2012

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

artículo científico publicado en 2009

Systematic interaction network filtering identifies CRMP1 as a novel suppressor of huntingtin misfolding and neurotoxicity

artículo científico publicado en 2015

TBK1 phosphorylates mutant Huntingtin and suppresses its aggregation and toxicity in Huntington's disease models

artículo científico publicado en 2020

TR-FRET-based duplex immunoassay reveals an inverse correlation of soluble and aggregated mutant huntingtin in huntington's disease

artículo científico publicado en 2012

Targeting H3K4 trimethylation in Huntington disease

artículo científico publicado en 2013

The Hdh(Q150/Q150) knock-in mouse model of HD and the R6/2 exon 1 model develop comparable and widespread molecular phenotypes

artículo científico publicado en 2006

The Huntington's disease candidate region exhibits many different haplotypes

artículo científico publicado en 1992

The Huntington's disease-related cardiomyopathy prevents a hypertrophic response in the R6/2 mouse model

artículo científico publicado en 2014

The PDE1/5 Inhibitor SCH-51866 Does Not Modify Disease Progression in the R6/2 Mouse Model of Huntington's Disease

artículo científico publicado en 2014

The S/T-Rich Motif in the DNAJB6 Chaperone Delays Polyglutamine Aggregation and the Onset of Disease in a Mouse Model.

artículo científico publicado en 2016

The human homeobox gene HOX7 maps to chromosome 4p16.1 and may be implicated in Wolf-Hirschhorn syndrome.

artículo científico publicado en 1990

The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease

artículo científico publicado en 2011

The new science of ageing

artículo científico publicado en 2015

The new science of ageing

artículo científico publicado en 2011

The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patients

artículo científico publicado en 2017

The polyubiquitin Ubc gene modulates histone H2A monoubiquitylation in the R6/2 mouse model of Huntington's disease

artículo científico publicado en 2009

The telomeric 60 kb of chromosome arm 4p is homologous to telomeric regions on 13p, 15p, 21p, and 22p.

artículo científico publicado en 1992

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

artículo científico publicado en 2009

Transgenic models of Huntington's disease

artículo científico publicado en 1997

Transgenic mouse models of neurodegenerative disease caused by CAG/polyglutamine expansions

artículo científico publicado en 1997

Treating the whole body in Huntington's disease

artículo científico publicado en 2015

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome

artículo científico publicado en 2016

Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice

artículo científico publicado en 1999

Use of high-content imaging to quantify transduction of AAV-PHP viruses in the brain following systemic delivery

artículo científico publicado en 2021

mHTT Seeding Activity: A Marker of Disease Progression and Neurotoxicity in Models of Huntington’s Disease

scholarly article by Anne Ast published in September 2018

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