Gillian Bates | Academic Influence

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Gillian Bates

Biology

#4431

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#6603

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Genetics

#912

World Rank

#1008

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Biology

Gillian Bates's Degrees

Bachelors Biology University of Oxford

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According to Wikipedia, Gillian Patricia Bates FMedSci FRS is a British biologist. She is distinguished for her research into the molecular basis of Huntington's disease and in 1998 was awarded the GlaxoSmithKline Prize as a co-discoverer of the cause of this disease. As of 2016, she is Professor of Neurogenetics at UCL Institute of Neurology and the co-director of UCL Huntington's Disease Centre.

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Gillian Bates's Published Works

Number of citations in a given year to any of this author's works

Total number of citations to an author for the works they published in a given year. This highlights publication of the most important work(s) by the author

Published Works

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes (1993) (7826)
Exon 1 of the HD Gene with an Expanded CAG Repeat Is Sufficient to Cause a Progressive Neurological Phenotype in Transgenic Mice (1996) (3026)
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. (1997) (2717)
Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation (1997) (2223)
Huntingtin-Encoded Polyglutamine Expansions Form Amyloid-like Protein Aggregates In Vitro and In Vivo (1997) (1272)
The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. (2000) (1068)
Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease (2003) (853)
Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology. (1999) (673)
Huntington disease (2015) (580)
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease (2008) (559)
A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis (1998) (545)
Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene. (1998) (527)
Global changes to the ubiquitin system in Huntington's disease (2007) (503)
Huntingtin and the molecular pathogenesis of Huntington's disease (2004) (485)
Huntingtin aggregation and toxicity in Huntington's disease (2003) (482)
Intranuclear Neuronal Inclusions in Huntington's Disease and Dentatorubral and Pallidoluysian Atrophy: Correlation between the Density of Inclusions andIT15CAG Triplet Repeat Length (1998) (443)
Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease. (2000) (441)
Selective Discrimination Learning Impairments in Mice Expressing the Human Huntington's Disease Mutation (1999) (405)
Abnormal Synaptic Plasticity and Impaired Spatial Cognition in Mice Transgenic for Exon 1 of the Human Huntington's Disease Mutation (2000) (401)
Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease (2013) (395)
IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome (2009) (369)
Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse (2000) (347)
Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach. (2004) (334)
A candidate for the cystic fibrosis locus isolated by selection for methylation-free islands (1987) (331)
Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation (1997) (324)
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. (2007) (322)
SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis (2010) (319)
Formation of polyglutamine inclusions in non-CNS tissue. (1999) (299)
Impaired Glutamate Uptake in the R6 Huntington's Disease Transgenic Mice (2001) (298)
Proteolysis of Mutant Huntingtin Produces an Exon 1 Fragment That Accumulates as an Aggregated Protein in Neuronal Nuclei in Huntington Disease* (2010) (292)
Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models (2009) (292)
Dysfunction of the Cholesterol Biosynthetic Pathway in Huntington's Disease (2005) (276)
Environmental enrichment slows disease progression in R6/2 Huntington's disease mice (2002) (270)
A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo. (2005) (269)
Structure and expression of the Huntington's disease gene: Evidence against simple inactivation due to an expanded CAG repeat (1994) (269)
Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease. (1999) (249)
SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates. (1998) (232)
UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome (2016) (227)
Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates. (2007) (220)
Histone deacetylase inhibitors as therapeutics for polyglutamine disorders (2006) (214)
Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice. (1999) (211)
DNA instability in postmitotic neurons (2008) (192)
Polyglutamine expansion of huntingtin impairs its nuclear export (2005) (189)
The huntingtin interacting protein HIP1 is a clathrin and alpha-adaptin-binding protein involved in receptor-mediated endocytosis. (2001) (180)
History of genetic disease: The molecular genetics of Huntington disease — a history (2005) (178)
Exendin-4 Improves Glycemic Control, Ameliorates Brain and Pancreatic Pathologies, and Extends Survival in a Mouse Model of Huntington's Disease (2009) (169)
Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions? (1998) (168)
The Hdh Q150/Q150 knock-in mouse model of HD and the R6/2 exon 1 model develop comparable and widespread molecular phenotypes (2007) (168)
Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease. (2011) (165)
Minocycline and doxycycline are not beneficial in a model of Huntington's disease (2003) (165)
Biomarkers for neurodegenerative diseases (2005) (165)
The Huntington's disease candidate region exhibits many different haplotypes (1992) (165)
Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease (2009) (160)
Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice (2006) (147)
SAHA Decreases HDAC 2 and 4 Levels In Vivo and Improves Molecular Phenotypes in the R6/2 Mouse Model of Huntington's Disease (2011) (147)
The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington’s disease patients (2017) (145)
HDAC4 Reduction: A Novel Therapeutic Strategy to Target Cytoplasmic Huntingtin and Ameliorate Neurodegeneration (2013) (145)
Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mouse model of Huntington's disease. (2006) (144)
HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation. (2014) (141)
Formation of Polyglutamine Inclusions in a Wide Range of Non-CNS Tissues in the HdhQ150 Knock-In Mouse Model of Huntington's Disease (2009) (141)
Standardization and statistical approaches to therapeutic trials in the R6/2 mouse (2003) (139)
Transgenic models of Huntington's disease. (1999) (137)
Targeting H3K4 trimethylation in Huntington disease (2013) (136)
Increased metabolism in the R6/2 mouse model of Huntington’s disease (2008) (134)
The S/T-Rich Motif in the DNAJB6 Chaperone Delays Polyglutamine Aggregation and the Onset of Disease in a Mouse Model. (2016) (132)
Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression. (2012) (130)
Sequence comparison of human and yeast telomeres identifies structurally distinct subtelomeric domains. (1997) (130)
Comparative sequence analysis of the human and pufferfish Huntington's disease genes (1995) (130)
SIRT2 Ablation Has No Effect on Tubulin Acetylation in Brain, Cholesterol Biosynthesis or the Progression of Huntington's Disease Phenotypes In Vivo (2012) (128)
Treating the whole body in Huntington's disease (2015) (122)
Suppression of protein aggregation by chaperone modification of high molecular weight complexes (2012) (121)
Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease. (2005) (117)
Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington’s disease (2007) (115)
Trinucleotide repeat expansions and human genetic disease. (1994) (112)
Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington's disease (HD) transgenic mice and HD patients. (2001) (112)
A brain-permeable small molecule reduces neuronal cholesterol by inhibiting activity of sirtuin 2 deacetylase. (2011) (109)
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 (2003) (109)
Proteasome impairment does not contribute to pathogenesis in R6/2 Huntington's disease mice: exclusion of proteasome activator REGgamma as a therapeutic target. (2006) (107)
Metabolic characterization of the R6/2 transgenic mouse model of Huntington's disease by high-resolution MAS 1H NMR spectroscopy. (2006) (107)
The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease. (2011) (105)
Hdac6 Knock-Out Increases Tubulin Acetylation but Does Not Modify Disease Progression in the R6/2 Mouse Model of Huntington's Disease (2011) (100)
SUMO-2 and PIAS1 Modulate Insoluble Mutant Huntingtin Protein Accumulation (2013) (96)
A cosmid contig and high resolution restriction map of the 2 megabase region containing the Huntington's disease gene (1993) (95)
Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation. (2007) (91)
Amyloid-like inclusions in Huntington’s disease (2000) (89)
A novel G protein-coupled receptor kinase gene cloned from 4p16.3. (1992) (85)
Dysfunction of the CNS-Heart Axis in Mouse Models of Huntington's Disease (2014) (85)
Alterations in the Mouse and Human Proteome Caused by Huntington’s Disease* (2002) (82)
From neuronal inclusions to neurodegeneration: neuropathological investigation of a transgenic mouse model of Huntington's disease. (1999) (82)
Abnormal Phosphorylation of Synapsin I Predicts a Neuronal Transmission Impairment in the R6/2 Huntington's Disease Transgenic Mice (2002) (79)
Inhibition of Polyglutamine Aggregation in R6/2 HD Brain Slices—Complex Dose–Response Profiles (2001) (78)
FAN1 modifies Huntington’s disease progression by stabilizing the expanded HTT CAG repeat (2018) (78)
Isolation of a further anonymous informative DNA sequence from chromosome seven closely linked to cystic fibrosis. (1986) (76)
Early and transient alteration of adenosine A2A receptor signaling in a mouse model of Huntington disease (2006) (76)
A Large Number of Protein Expression Changes Occur Early in Life and Precede Phenotype Onset in a Mouse Model for Huntington Disease*S (2009) (76)
Complex patterns of linkage disequilibrium in the Huntington disease region. (1991) (75)
Gastrointestinal dysfunction contributes to weight loss in Huntington's disease mice (2011) (75)
Transgenic Mice in the Study of Polyglutamine Repeat Expansion Diseases (1998) (74)
TR-FRET-based duplex immunoassay reveals an inverse correlation of soluble and aggregated mutant huntingtin in huntington's disease. (2012) (73)
Frequency of nuclear mutant huntingtin inclusion formation in neurons and glia is cell‐type‐specific (2016) (71)
Defined physical limits of the Huntington disease gene candidate region. (1991) (71)
Striking changes in anxiety in Huntington's disease transgenic mice (1998) (71)
Genetic Knock-Down of HDAC7 Does Not Ameliorate Disease Pathogenesis in the R6/2 Mouse Model of Huntington's Disease (2009) (70)
Evaluation of the benzothiazole aggregation inhibitors riluzole and PGL-135 as therapeutics for Huntington's disease (2006) (70)
Reduction of GnRH and infertility in the R6/2 mouse model of Huntington's disease (2005) (69)
Partial resistance to malonate‐induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length (2001) (65)
The Ubiquitin-Proteasome Reporter GFPu Does Not Accumulate in Neurons of the R6/2 Transgenic Mouse Model of Huntington's Disease (2009) (64)
Transgenic models of Huntington's disease. (1997) (63)
HDAC4-Myogenin Axis As an Important Marker of HD-Related Skeletal Muscle Atrophy (2015) (61)
Regional localization of the gene coding for human brain nitric oxide synthase (NOS1) to 12q24.2-->24.31 by fluorescent in situ hybridization. (1993) (61)
A yeast artificial chromosome telomere clone spanning a possible location of the Huntington disease gene. (1990) (61)
Experimental therapeutics in Huntington's disease: are models useful for therapeutic trials? (2003) (60)
Amyloid, prions, and other protein aggregates (2000) (60)
Genetic Knock-Down of Hdac3 Does Not Modify Disease-Related Phenotypes in a Mouse Model of Huntington's Disease (2012) (59)
Identification of an HD patient with a (CAG)180 repeat expansion and the propagation of highly expanded CAG repeats in lambda phage (1997) (59)
Inclusion formation in Huntington's disease R6/2 mouse muscle cultures (2003) (58)
Characterization of a yeast artificial chromosome contig spanning the Huntington's disease gene candidate region (1992) (56)
The organization of the human complement factor I gene (IF): a member of the serine protease gene family. (1994) (55)
Optimisation of region-specific reference gene selection and relative gene expression analysis methods for pre-clinical trials of Huntington's disease (2008) (55)
A common gene expression signature in Huntington’s disease patient brain regions (2014) (55)
A human single-chain Fv intrabody preferentially targets amino-terminal huntingtin fragments in striatal models of Huntington's disease (2005) (54)
Microdissection of and microcloning from the short arm of human chromosome 2 (1986) (53)
Correlations of Behavioral Deficits with Brain Pathology Assessed through Longitudinal MRI and Histopathology in the R6/2 Mouse Model of HD (2013) (52)
Aberrantly spliced HTT, a new player in Huntington’s disease pathogenesis (2013) (51)
Characterisation of immune cell function in fragment and full-length Huntington's disease mouse models (2014) (51)
Arfaptin 2 regulates the aggregation of mutant huntingtin protein (2002) (50)
A zinc-finger gene ZNF141 mapping at 4p16.3/D4S90 is a candidate gene for the Wolf-Hirschhorn (4p-) syndrome. (1993) (48)
Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies (2014) (47)
Downregulation of cannabinoid receptor 1 from neuropeptide Y interneurons in the basal ganglia of patients with Huntington's disease and mouse models (2013) (46)
Stall in Canonical Autophagy-Lysosome Pathways Prompts Nucleophagy-Based Nuclear Breakdown in Neurodegeneration (2017) (45)
mHTT Seeding Activity: A Marker of Disease Progression and Neurotoxicity in Models of Huntington's Disease. (2018) (44)
Regulatory mechanisms of incomplete huntingtin mRNA splicing (2018) (44)
Mapping of cosmid clones in Huntington's disease region of chromosome 4 (1991) (42)
HAP1‐huntingtin interactions do not contribute to the molecular pathology in Huntington's disease transgenic mice (1998) (40)
Implantation of undifferentiated and pre-differentiated human neural stem cells in the R6/2 transgenic mouse model of Huntington’s disease (2012) (40)
Correlations of Behavioral Deficits with Brain Pathology Assessed through Longitudinal MRI and Histopathology in the R6/1 Mouse Model of Huntington’s Disease (2013) (39)
Depletion of rabphilin 3A in a transgenic mouse model (R6/1) of Huntington's disease, a possible culprit in synaptic dysfunction (2005) (39)
Juvenile huntington's disease and other trinucleotide repeat disorders (2009) (38)
Oral Administration of the Pimelic Diphenylamide HDAC Inhibitor HDACi 4b Is Unsuitable for Chronic Inhibition of HDAC Activity in the CNS In Vivo (2012) (38)
The human homeobox gene HOX7 maps to chromosome 4p16.1 and may be implicated in Wolf-Hirschhorn syndrome (1990) (38)
Mapping DNA sequences in a human X-chromosome deletion which extends across the region of the Duchenne muscular dystrophy mutation. (1985) (38)
Cloning of the α–adducin gene from the Huntington's disease candidate region of chromosome 4 by exon amplification (1992) (37)
The telomeric 60 kb of chromosome arm 4p is homologous to telomeric regions on 13p, 15p, 21p, and 22p. (1992) (35)
CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease. (2010) (35)
Genetic Deletion of Transglutaminase 2 Does Not Rescue the Phenotypic Deficits Observed in R6/2 and zQ175 Mouse Models of Huntington's Disease (2014) (35)
Contesting the dogma of an age-related heat shock response impairment: implications for cardiac-specific age-related disorders (2014) (35)
The direct screening of cosmid libraries with YAC clones. (1991) (34)
A long-range restriction map encompassing the cystic fibrosis locus and its closely linked genetic markers. (1988) (33)
HDAC4 Does Not Act as a Protein Deacetylase in the Postnatal Murine Brain In Vivo (2013) (32)
Mouse Models of Huntington's Disease. (2018) (32)
Detection of polyglutamine aggregation in mouse models. (1999) (31)
Changes in GAD67 mRNA expression evidenced by in situ hybridization in the brain of R6/2 transgenic mice (2003) (30)
The Huntington's Disease-Related Cardiomyopathy Prevents a Hypertrophic Response in the R6/2 Mouse Model (2014) (29)
Loss of cortical and thalamic neuronal tenascin‐C expression in a transgenic mouse expressing exon 1 of the human Huntington disease gene (2001) (28)
SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington’s Disease Mice (2016) (28)
Transgenic mouse models of neurodegenerative disease caused by CAG/polyglutamine expansions. (1997) (28)
Phenotype onset in Huntington’s disease knock‐in mice is correlated with the incomplete splicing of the mutant huntingtin gene (2019) (27)
Meso scale discovery-based assays for the detection of aggregated huntingtin (2019) (26)
New DNA markers in the Huntington's disease gene candidate region (1991) (26)
The polyubiquitin Ubc gene modulates histone H2A monoubiquitylation in the R6/2 mouse model of Huntington's disease (2009) (26)
Intranuclear inclusions in subtypes of striatal neurons in Huntington's disease transgenic mice. (1999) (26)
Aberrant processing of the Fugu HD (FrHD) mRNA in mouse cells and in transgenic mice. (1997) (24)
Subcellular Localization And Formation Of Huntingtin Aggregates Correlates With Symptom Onset And Progression In A Huntington’S Disease Model (2020) (24)
Expanded glutamines and neurodegeneration--a gain of insight. (1996) (24)
Systematic interaction network filtering identifies CRMP1 as a novel suppressor of huntingtin misfolding and neurotoxicity (2015) (24)
TBK1 phosphorylates mutant Huntingtin and suppresses its aggregation and toxicity in Huntington's disease models (2020) (23)
Live-cell super-resolution microscopy reveals a primary role for diffusion in polyglutamine-driven aggresome assembly (2018) (23)
HSF1-dependent and -independent regulation of the mammalian in vivo heat shock response and its impairment in Huntington's disease mouse models (2017) (23)
Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 knock-in mouse model of Huntington’s disease (2012) (23)
Transgenic models of Huntington's disease. Hum Mol Genet 6:1633-1637 (1997) (22)
Fragments of HdhQ150 Mutant Huntingtin Form a Soluble Oligomer Pool That Declines with Aggregate Deposition upon Aging (2012) (22)
Polyglutamine expansion and Huntington's disease. (1998) (21)
Novel Isoforms of Heat Shock Transcription Factor 1, HSF1γα and HSF1γβ, Regulate Chaperone Protein Gene Transcription* (2014) (20)
Huntington's disease: Exploiting expression (2001) (20)
The polyglutamine diseases (2002) (19)
Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington’s disease mice (2017) (19)
MEF2 impairment underlies skeletal muscle atrophy in polyglutamine disease (2020) (18)
The isolation of cDNAs within the Huntington disease region by hybridisation of yeast artificial chromosomes to a cDNA library. (1993) (17)
Radiation hybrid map spanning the Huntington disease gene region of chromosome 4. (1992) (17)
One Misfolded Protein Allows Others to Sneak By (2006) (17)
Huntingtonâ€™s Disease (2014) (17)
Disruption to schizophrenia-associated gene Fez1 in the hippocampus of HDAC11 knockout mice (2017) (16)
FAN1 controls mismatch repair complex assembly via MLH1 retention to stabilize CAG repeat expansion in Huntington’s disease (2021) (16)
Expression of mutant exon 1 huntingtin fragments in human neural stem cells and neurons causes inclusion formation and mitochondrial dysfunction (2020) (16)
Further data supporting linkage between cystic fibrosis and the met oncogene and haplotype analysis with met and pJ3.11. (1986) (14)
Faculty Opinions recommendation of Enhanced neurofibrillary degeneration in transgenic mice expressing mutant tau and APP. (2001) (14)
Mouse models of triplet repeat diseases (2006) (14)
Correlations of Behavioral Deficits with Brain Pathology Assessed through Longitudinal MRI and Histopathology in the HdhQ150/Q150 Mouse Model of Huntington’s Disease (2017) (13)
In Vivo Profiling Reveals a Competent Heat Shock Response in Adult Neurons: Implications for Neurodegenerative Disorders (2015) (12)
RNA Related Pathology in Huntington's Disease. (2018) (12)
Erratum: SIRT1 Activity is linked to its brain region-specific phosphorylation and is impaired in huntington's disease mice (PLoS ONE (2016) 11:1 (e0145425) DOI: 10.1371/journal.pone.0145425) (2016) (12)
Mouse models of triplet repeat diseases. (2006) (11)
Reducing Igf-1r Levels Leads To Paradoxical and Sexually Dimorphic Effects in HD Mice (2014) (11)
Uninterrupted CAG repeat drives striatum-selective transcriptionopathy and nuclear pathogenesis in human Huntingtin BAC mice (2022) (11)
In vivo neutralization of the protagonist role of macrophages during the chronic inflammatory stage of Huntington’s disease (2018) (11)
Neuropsychiatry and Neuropsychology (2014) (11)
Silencing Srsf6 does not modulate incomplete splicing of the huntingtin gene in Huntington’s disease models (2020) (11)
Characterization of Gastric Mucosa Biopsies Reveals Alterations in Huntington's Disease (2015) (11)
Inhibition of tumour necrosis factor alpha in the R6/2 mouse model of Huntington’s disease by etanercept treatment (2019) (11)
Ablation of kynurenine 3-monooxygenase rescues plasma inflammatory cytokine levels in the R6/2 mouse model of Huntington’s disease (2021) (10)
Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease (2016) (10)
The PDE1/5 Inhibitor SCH-51866 Does Not Modify Disease Progression in the R6/2 Mouse Model of Huntington’s Disease (2014) (10)
Huntington's disease: In reverse gear (2000) (9)
Extensive Expression Analysis of Htt Transcripts in Brain Regions from the zQ175 HD Mouse Model Using a QuantiGene Multiplex Assay (2019) (9)
Cystic fibrosis linkage exclusion data. (1986) (9)
Transgenic Mouse Models of Huntington’s Disease (2000) (8)
False-negative result for Huntington's disease mutation (1994) (8)
HSF 1-dependent and-independent regulation of the mammalian in vivo heat shock response and its impairment in Huntington ' s disease mouse models (2017) (7)
Small, Seeding-Competent Huntingtin Fibrils Are Prominent Aggregate Species in Brains of zQ175 Huntington’s Disease Knock-in Mice (2021) (7)
Monitoring aggregate formation in organotypic slice cultures from transgenic mice. (2004) (7)
Development of novel bioassays to detect soluble and aggregated Huntingtin proteins on three technology platforms (2021) (6)
M09 Myostatin Inhibition as a Novel Approach to Targeting Muscle Pathology in HD (2014) (6)
Distribution of trinucleotide repeat sequences across a 2 Mbp region containing the Huntington's disease gene. (1994) (6)
Transcript map of the human chromosome 4p16.3 consisting of 627 cDNA clones derived from 1 Mb of the Huntington's disease locus. (1996) (6)
Hunting in the calm before the storm (2000) (6)
Exon trapping and sequence-based methods of gene finding in transcript mapping of human 4p 16.3 (1997) (5)
Use of high-content imaging to quantify transduction of AAV-PHP viruses in the brain following systemic delivery (2021) (5)
HAP 1-huntingtin interactions do not contribute to the molecular pathology in Huntington ' s disease transgenic mice (1998) (5)
Biochemical and genetic exclusion of calmodulin as the site of the basic defect in cystic fibrosis (1987) (5)
Correlative light and electron microscopy suggests that mutant huntingtin dysregulates the endolysosomal pathway in presymptomatic Huntington’s disease (2021) (5)
Current protocols in human genetics: edited by Nicholas C. Dracopoli et al. Current Protocols, 1994. $295.00 loose leaf (750 pages) ISBN 0 471 03420 7 (1995) (4)
Generation of high-density DNA markers from yeast artificial chromosome DNA by single unique primer-polymerase chain reaction. (1993) (4)
Isolation of YAC ends by plasmid rescue. (1996) (4)
A10 SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis (2010) (4)
Murine models of Huntington's Disease (1998) (4)
Juvenile Huntington’s disease and mouse models of Huntington’s disease (2009) (3)
The heat shock response, determined by QuantiGene multiplex, is impaired in HD mouse models and not caused by HSF1 reduction (2021) (3)
B38 The effect of Hdac4 reduction post-weaning on hd-related phenotypes in R6/2 mice (2016) (3)
Chapter 14 - Molecular Pathogenesis and Therapeutic Targets in Huntington's Disease (2006) (2)
Molecular Approaches to identify the cystic fibrosis gene defect (1987) (2)
TBK1 regulates autophagic clearance of soluble mutant huntingtin and inhibits aggregation/toxicity in different models of Huntington’s disease (2019) (2)
The application of molecular genetics to the study of the basic defect causing cystic fibrosis. (1987) (2)
In vivo magnetic resonance studies in a transgenic mouse model of Huntington's disease. (2002) (2)
Genetic deletion of S6k1 does not rescue the phenotypic deficits observed in the R6/2 mouse model of Huntington’s disease (2019) (2)
University of Dundee UBQLN 2 mediates autophagy-independent protein aggregate clearance by the proteasome (2)
Alternative processing of human HTT mRNA with implications for Huntington’s disease therapeutics (2022) (2)
P3-377: A novel pathogenic pathway of immune activation detectable before cinical onset in Huntington's disease (2008) (1)
Transglutaminase 6 Is Colocalized and Interacts with Mutant Huntingtin in Huntington Disease Rodent Animal Models (2021) (1)
Treatment Efficacy of Transplanted Human Striatal Neural Stem/Progenitor Cells in the R6/2 Mouse Model of Huntington's Disease (2010) (1)
A Comprehensive, Multidisciplinary Description of the Progressive Pathology Exhibited by the R6/2 Mouse Model of Huntington's Disease (2010) (1)
Genetic knock-down of HDAC 4 improves motor impairment in the R6/2 mouse model of Huntington's disease (2008) (1)
D21 Analysis of blood and CSF biomarkers in mouse models of Huntington’s disease (2022) (1)
RFLP for D4S12, an anonymous single copy genomic clone at 4pter-4q26 [HGM8 provisional no. D4S12]. (1985) (1)
Molecular genetics and the basic defect causing cystic fibrosis. (1986) (1)
B23 Immune dysfunction in HD human myeloid cells is caused by NFκB pathway dysregulation and is reversed by lowering HTT levels (2012) (1)
B44 The Cns-heart Axis Is A Source Of Cardiac Dysfunction In Mouse Models Of Huntington’s Disease (2014) (1)
The Huntington disease gene--still a needle in a haystack? (1993) (1)
Molecular Pathology of Huntington's Disease: Animal Models and Nuclear Mechanisms (1999) (1)
Inhibition of tumour necrosis factor alpha in the R6/2 mouse model of Huntington’s disease by etanercept treatment (2019) (0)
Ex vivo 100 μm isotropic diffusion MRI‐based tractography of connectivity changes in the end‐stage R6/2 mouse model of Huntington's disease (2022) (0)
short arm of human chromosome 2. Microdissection of and microcloning from the (2013) (0)
B27 Abnormal bioenergetics in inclusion-containing mutant HTT exon 1 primary human neurons (2016) (0)
Generation and characterisation of mouse embryonic stem cell lines for the study of HuntingtonG (2011) (0)
A31 The development of translational biomarkers of neuroinflammation in a mouse model of huntington’s disease (2018) (0)
Dietary creatine supplementation in a mouse model of Huntington's disease. (2002) (0)
F05 Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression (2012) (0)
Correction: SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice (2016) (0)
Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington’s disease mice (2017) (0)
Neurobiology of Disease Dysfunction of the Cholesterol Biosynthetic Pathway in Huntington ’ s Disease (0)
Combined Proteome and Transcriptome Analysis of Alterations in the Mouse Brain Caused by Huntington?s Disease (2005) (0)
Early detection of exon 1 huntingtin aggregation in zQ175 brains by molecular and histological approaches (2023) (0)
A03 Alternative processing of human HTT MRNA in YAC128 mice: implications for Huntington’s disease therapeutics (2022) (0)
A04 The role of splicing factor SRSF6 in incomplete splicing of the HTT transcript (2018) (0)
A large number of protein expression changes occur early in life and precede phenotype onset in different animal models for Huntington's disease (2009) (0)
B8 Ablation of p62 modulates levels of soluble and aggregated mutant huntingtin and delays end-stage disease in R6/2 mice (2016) (0)
B3 Comparison of the effect of a pure CAG repeat and mixed cagcaa repeat on the extent to which the htt gene is aberrantly spliced in knock-in mice (2016) (0)
L3 Systemic administration of a novel AAV variant results in widespread and efficient gene transfer in R6/2 mice (2016) (0)
Regulatory mechanisms of incomplete huntingtin mRNA splicing (2018) (0)
A03 Regulatory mechanisms of incomplete HTT MRNA splicing in huntington’s disease (2018) (0)
B6 Super-resolution fluorescence imaging of the seeding and polymerizatoin of the huntingtin exon 1 protein (2016) (0)
Model depicting the mechanism by which mutant huntingtin causes cardiac dysfunction in murine HD hearts. (2014) (0)
A21 Transcriptional dysregulation is caused by the accumulation of detergent insoluble HTT aggregates in the nucleus (2018) (0)
B24 Assessment of immune system activation status during the course of disease in huntington’s disease mouse model (2016) (0)
A16 The temporal and spatial appearance of huntingtin aggregates in the brains of the ZQ175 mouse model of huntington’s disease (2018) (0)
B19 Development of in vitro models to investigate the pathogenesis of huntington’s disease and screen for therapeutic agents (2018) (0)
Disruption to schizophrenia-associated gene Fez1 in the hippocampus of HDAC11 knockout mice (2017) (0)
Reduction in PA28αβ activation in HD mouse brain correlates to increased mHTT aggregation in cell models (2022) (0)
B4 Detection of the aberrantly spliced exon 1 – intron 1 htt mRNA in HD patient post mortem brain tissue and fibroblast lines (2016) (0)
A18 Investigating the mechanisms of the heat shock response impairment in huntington’s disease (2018) (0)
A common gene expression signature in Huntington’s disease patient brain regions (2014) (0)
C02 FAN1 controls cag repeat expansion in huntington’s disease by dual functions, MLH1 retention and nuclease activity (2021) (0)
Identification of two novel heat shock factor isoforms - HSF1 isoform ratio regulates the level of transcription of heat shock protein genes.* (2014) (0)
Wiley Encyclopedia of Molecular Medicine Volume 3 (H-M) (2002) (0)
In vivo, longitudinal, 1H Magnetic Resonance Spectroscopy in a transgenic mouse model of Huntington's Disease (2002) (0)
D05 Development and optimisation of a quantigene assay to measure HTT transcripts levels and investigate the efficiency of lowering htt in vivo (2018) (0)
Erratum: Valenza et al., “Dysfunction of the Cholesterol Biosynthetic Pathway in Huntington's Disease” (2020) (0)
The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington’s disease patients (2017) (0)
FurtherDataSupporting LinkagebetweenCystic Fibrosis andthemetOncogene andHaplotype Analysis withmetandpJ3.11 (1986) (0)
RFLP for pHM20 (D2S12), an anonymous DNA sequence localised to 2p23-2pter. (1987) (0)
Experimental therapeutics in mouse models of Huntington's disease. (2005) (0)
D03 Novel bioassays to detect soluble and aggregated isoforms of the huntingtin protein on three technology platforms (2022) (0)
Progress towards identification of the gene defect in cystic fibrosis (1987) (0)
Intranuclear Neuronal Inclusions: A novel marker in Huntington's disease which correlates with CAG triplet repeat length (1998) (0)
In vivo neutralization of the protagonist role of macrophages during the chronic inflammatory stage of Huntington’s disease (2018) (0)
Mutant HTT seeding activity: a marker of disease progression and neurotoxicity in models of Huntington’s disease (2018) (0)
The Hunt in Huntington: What Causes Toxicity? (2009) (0)
The molecular basis of Huntington's disease and polyglutamine disorders (2000) (0)
HSF1-dependent and -independent regulation of the mammalian in vivo heat shock response and its impairment in Huntington's disease mouse models (2017) (0)
Analysis of the transgenome of MET transfectant cell lines reveals that MET activation is accompanied by an interstitial insertion (1990) (0)
Absolute Quantification of Brain metabolites in a Mouse model of Huntington's Disease (2003) (0)
The use of quantitative real-time PCR to characterise transcriptional dysregulation in mouse models of Huntington's disease (2008) (0)
A20 A role for transglutaminase 6 in hd pathology (2018) (0)
THE EFFECT OF HDAC 4 REDUCTION POST-WEANING ON HD-RELATED PHENOTYPES IN R 6 / 2 MICE (2019) (0)
Cloning of human telomeres in Saccharomyces cerevisiae. (1996) (0)
Transgenic models of HD (1997) (0)
Characterization of the protein component of Methanococcus jannaschii RNase P (2001) (0)
Molecular Approaches Toward the Isolation of the Huntington’s Disease Gene (1990) (0)
A11 The temporal and spatial appearance of HTT aggregation in the brains of zQ175 mice (2022) (0)
C01 R6/2 Mice With A 90q Repeat Expansion Show Earlier Aggregate Pathology In Brain (2014) (0)
A large number of protein expression changes in a mouse model of Huntington's disease early in life precede overt disease symptoms (2008) (0)
C02 Molecular Analysis Of The Zq175 Knock-in Mouse Model (2014) (0)
Correlative light and electron microscopy suggests that mutant huntingtin dysregulates the endolysosomal pathway in presymptomatic Huntington’s disease (2021) (0)
A17 Myeloid cell function in mouse models of Huntington's disease (2010) (0)
DEFINITION OF THE PHYSICAL AND GENETIC LIMITS OF THE HUNTINGTONS-DISEASE GENE CANDIDATE REGION AND PROGRESS TOWARDS ITS ISOLATION AS A YAC CONTIG (1991) (0)
Overall visit frequency detected in the PhenoCube system as a function of genotype, age and light cycle phase in animals from the zQ175×TG2 KO line. (2014) (0)
A12 HDAC4 interacts with huntington and HDAC4 reduction decreases cytoplamsic aggregation and rescues synaptic dysfunction in HD mouse models (2012) (0)
Erratum: Heber et al., “Mice with Combined Gene Knock-Outs Reveal Essential and Partially Redundant Functions of Amyloid Precursor Protein Family Members” (2020) (0)
B09 Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HDHQ150 knock-in mouse model of Huntington's disease (2012) (0)
Central nervous system diseases. (1999) (0)
Reply (2003) (0)
Correction: SIRT2 Ablation Has No Effect on Tubulin Acetylation in Brain, Cholesterol Biosynthesis or the Progression of Huntington’s Disease Phenotypes In Vivo (2021) (0)
D11 Expression Analysis Of Genes Coding For Pet Ligands In HD Mouse Models (2014) (0)
D08 A Holistic Network Analysis Of Gene Expression Data In Huntington’s Disease Patients Reveals A Common Signature Of Transcriptional Dysregulation (2014) (0)
Absolute quantification of metabolites using a surface coil and an external reference in mouse brain. (2002) (0)
B03 Analysis of a Huntington’s disease knock-in mouse model designed to prevent the generation of the exon 1 HTT protein (2022) (0)
A19 Peripheral Pathologies in Mouse Models of HD and the Inhibtion of Myostatin Signalling as a Possible Therapeutic Target (2014) (0)
Implantation of undifferentiated and pre-differentiated human neural stem cells in the R6/2 transgenic mouse model of Huntington’s disease (2012) (0)
A4 Development of disease modifying therapies in Huntington's disease (2012) (0)
Dynamics of somatic CAG instability in Huntington's disease. (2005) (0)
B10 Inclusion formation in mutant HTT exon 1 expressing human neuronal cells (2016) (0)
B17 Establishing a neural progenitor cell model of huntington’s disease (2018) (0)
Huntington's disease: molecular pathogenesis and therapeutic approaches. (2006) (0)
Chronic administration of isoproterenol causes a significant transcriptional deregulation of many Hdacs and Sirtuins. (2014) (0)
Insights into the molecular pathogenesis of Huntington's disease (2001) (0)
Correlative light and electron microscopy reveals that mutant huntingtin dysregulates the endolysosomal pathway in presymptomatic Huntington’s disease (2021) (0)
PhysiologyDavid and Wolfgang-Michael Franz From Pluripotency to Distinct Cardiomyocyte Subtypes (2015) (0)
In vivo MR studies in a transgenic mouse model of Huntington's disease (2001) (0)

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