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List of works by Gillian Bates

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

scientific article

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

scientific article published in Nature

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

scientific article

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

scientific article

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

scientific article

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

scientific article

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

scientific article

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

scientific article

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

scientific article published on November 1997

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

scientific article published on 22 January 2013

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

scientific article published on 11 October 2013

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

scientific article

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

scientific article (publication date: 26 September 1997)

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

scientific article

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

scientific article

Amyloid-like inclusions in Huntington's disease.

scientific article published in January 2000

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

scientific article published in January 1998

Arfaptin 2 regulates the aggregation of mutant huntingtin protein

scientific article (publication date: March 2002)

BIOMEDICINE: One Misfolded Protein Allows Others to Sneak By.

scientific article published in March 2006

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

scientific article published on July 1987

Biomarkers for neurodegenerative diseases

scientific article

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

scientific article published in April 1999

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

scientific article

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

scientific article

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

scientific article published in September 2003

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

scientific article

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

scientific article published on 26 June 2015

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

scientific article published on November 1, 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

scientific article published in December 2003

Complex patterns of linkage disequilibrium in the Huntington disease region ⬇️

scientific article published on October 1, 1991

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

scientific article

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

scientific article published on 23 September 2005

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

scientific article published on 26 February 2016

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

scientific article

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

scientific article

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

scientific article

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

scientific article published on 14 April 2021

DNA instability in postmitotic neurons

scholarly article

Defined physical limits of the Huntington disease gene candidate region ⬇️

scientific article published on July 1, 1991

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

scientific article published on 20 June 2005

Detection of polyglutamine aggregation in mouse models

scientific article published in January 1999

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

scientific article published on 19 September 2017

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

scientific article published on 21 November 2012

Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies

scientific article published on 26 November 2013

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

scientific article

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

scientific article published in October 2005

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

scientific article

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

scientific article

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

scientific article

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

scientific article published on 18 August 2005

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

scientific article

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

scientific article (publication date: November 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

scientific article published on March 1996

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

scientific article

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

scientific article (publication date: 8 August 1997)

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

scientific article

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

scientific article

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

scientific article published on 12 September 2016

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

scientific article

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

scientific article published in January 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

scientific article (publication date: 2012)

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

scientific article

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

scientific article

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

scientific article (publication date: 2013)

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

scientific article (publication date: November 2013)

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

scientific article (publication date: March 2015)

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

scientific article published on 2 October 2017

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

scientific article published on 22 January 2014

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

scientific article (publication date: 2011)

Histone deacetylase inhibitors as therapeutics for polyglutamine disorders

scientific article published on October 2006

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

scientific article published in October 2005

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

scientific article

Hunting in the calm before the storm

scientific article published in August 2000

Huntingtin

Huntingtin aggregation and toxicity in Huntington's disease ⬇️

scientific article (publication date: 10 May 2003)

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

scientific article

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

scientific article published in August 1997

Huntington disease

Huntington's disease. Exploiting expression

scientific article

Huntington's disease. In reverse gear

scientific article published in April 2000

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

scientific article

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

scientific article published on January 2010

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

scientific article published in May 1997

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

scientific article

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

scientific article

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

scientific article published on 2 July 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

scientific article published in October 2003

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

scientific article

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

scientific article published in December 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.

scientific article published in February 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

scientific article published in April 1998

Isolation of YAC Ends by Plasmid Rescue

scientific article published on 01 January 1996

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

scientific article published in Journal of Biological Chemistry

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

scientific article published in February 2001

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

scientific article published on 26 March 2019

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

scientific article published in March 2006

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

scientific article published in August 2003

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

scientific article published in January 2000

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

scientific article published in January 2000

Monitoring aggregate formation in organotypic slice cultures from transgenic mice

scientific article published in January 2004

Mouse Models of Huntington's Disease

scientific article published on 01 January 2018

Mouse Models of Triplet Repeat Diseases

scientific article published on 01 February 2006

Mouse models of triplet repeat diseases

scientific article published on January 2004

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

scientific article published on 17 September 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

scientific article

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

scientific article

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

scientific article published on September 1, 1991

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

scientific article

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

scientific article

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

scientific article published on 27 October 2008

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

scientific article

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

scientific article published in August 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

scientific article

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

scientific article published on 13 April 2006

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

scientific article published on 28 April 2004

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

scientific article published on 25 November 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

scientific article

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

scientific article

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

scientific article

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

scientific article published on January 1987

RNA Related Pathology in Huntington's Disease

scientific article published in January 2018

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

scientific article published on August 1992

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

scientific article

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

scientific article published in September 2005

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

scientific article published in January 1993

Regulatory mechanisms of incomplete huntingtin mRNA splicing

scientific article published in Nature Communications

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

scientific article (publication date: 2011)

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

scientific article published on 27 January 2016

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

scientific article

SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis

scientific article

SUMO-2 and PIAS1 modulate insoluble mutant huntingtin protein accumulation

scientific article published on 18 July 2013

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

scientific article

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

scientific article

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

scientific article published in August 1997

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

scientific article published on 20 August 2020

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

scientific article published on 17 November 2017

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

scientific article published on September 2003

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

scientific article published in November 1998

Striking changes in anxiety in Huntington's disease transgenic mice

scientific article published in September 1998

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

scientific article

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

scholarly article

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

scientific article published on 6 March 2012

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

scientific article

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

scientific article published on May 2015

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

scientific article published on 05 August 2020

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

scientific article

Targeting H3K4 trimethylation in Huntington disease

scientific article published on 19 July 2013

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

scientific article

The Huntington's disease candidate region exhibits many different haplotypes

scientific article (publication date: May 1992)

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

scientific article

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

scientific article published on 13 February 2014

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

scientific article published on 12 April 2016

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

scientific article

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

scientific article

The new science of ageing

scientific article published on September 2015

The new science of ageing

scientific article published on January 2011

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

scientific article

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

scientific article

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

scientific article

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

scientific article

Transgenic Mice in the Study of Polyglutamine Repeat Expansion Diseases ⬇️

scientific article published on October 1, 1998

Transgenic models of Huntington's disease

scientific article published on January 1997

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

scientific article published on November 1997

Treating the whole body in Huntington's disease

scientific article

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome

scientific article published on 27 July 2016

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

scientific article published in July 1999

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

scientific article published on 17 May 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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