Sarah Tabrizi
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British neurologist and neuroscientist
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Why Is Sarah Tabrizi Influential?
(Suggest an Edit or Addition)According to Wikipedia, Sarah Joanna Tabrizi FMedSci is a British neurologist and neuroscientist in the field of neurodegeneration, particularly Huntington's disease. She is a Professor and Joint Head of the Department of Neurodegenerative Diseases at the UCL Institute of Neurology; the founder and Director of the UCL Huntington's Disease Centre; a Principal Investigator at the UK Dementia Research Institute at UCL; and an Honorary Consultant Neurologist at the National Hospital for Neurology and Neurosurgery, Queen Square, London, where she established the Multidisciplinary Huntington's Disease Clinic. The UCL Huntington’s Disease Centre was officially opened on 1 March 2017 by UCL President and Provost Professor Michael Arthur.
Sarah Tabrizi'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
- Huntington's disease: from molecular pathogenesis to clinical treatment (2011) (1378)
- Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data (2009) (872)
- Huntington disease: natural history, biomarkers and prospects for therapeutics (2014) (754)
- Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data (2013) (683)
- PINK1-Associated Parkinson's Disease Is Caused by Neuronal Vulnerability to Calcium-Induced Cell Death (2009) (614)
- Huntington disease (2015) (580)
- A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease (2008) (559)
- Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis (2011) (530)
- Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data (2012) (472)
- Huntington's disease: a clinical review (2018) (405)
- Targeting Huntingtin Expression in Patients with Huntington's Disease. (2019) (393)
- Microglial activation in presymptomatic Huntington's disease gene carriers. (2005) (382)
- Proteome-based plasma biomarkers for Alzheimer's disease. (2006) (380)
- PINK1 Is Necessary for Long Term Survival and Mitochondrial Function in Human Dopaminergic Neurons (2008) (311)
- Large C9orf72 hexanucleotide repeat expansions are seen in multiple neurodegenerative syndromes and are more frequent than expected in the UK population. (2013) (309)
- Mitochondria in the etiology and pathogenesis of parkinson's disease (1998) (254)
- Disease-associated prion protein oligomers inhibit the 26S proteasome. (2007) (251)
- Neurofilament light protein in blood as a potential biomarker of neurodegeneration in Huntington's disease: a retrospective cohort analysis (2017) (224)
- Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates. (2007) (220)
- Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study (2017) (208)
- Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study (2017) (208)
- Rapid eye movement sleep disturbances in Huntington disease. (2008) (205)
- Therapies targeting DNA and RNA in Huntington's disease (2017) (204)
- Prevalence of adult Huntington's disease in the UK based on diagnoses recorded in general practice records (2013) (192)
- Observing Huntington’s Disease: the European Huntington’s Disease Network’s REGISTRY (2011) (186)
- Expression of mutant α-synuclein causes increased susceptibility to dopamine toxicity (2000) (186)
- Observing Huntington’s Disease: the European Huntington’s Disease Network’s REGISTRY (2011) (186)
- Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients. (2015) (185)
- An event-based model for disease progression and its application in familial Alzheimer's disease and Huntington's disease (2012) (173)
- Gene expression in Huntington's disease skeletal muscle: a potential biomarker. (2005) (173)
- DNA repair pathways underlie a common genetic mechanism modulating onset in polyglutamine diseases (2016) (172)
- Huntington’s disease (2010) (170)
- Microglial activation in regions related to cognitive function predicts disease onset in Huntington's disease: A multimodal imaging study (2011) (169)
- Hsa-miR-34b is a plasma-stable microRNA that is elevated in pre-manifest Huntington's disease. (2011) (168)
- C9orf72 expansions are the most common genetic cause of Huntington disease phenocopies (2014) (168)
- Hypothalamic involvement in Huntington's disease: an in vivo PET study. (2008) (167)
- Biomarkers for neurodegenerative diseases (2005) (165)
- White matter connections reflect changes in voluntary-guided saccades in pre-symptomatic Huntington's disease. (2008) (164)
- The Prevalence of Huntington's Disease (2016) (155)
- Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities (2020) (154)
- Huntingtin Lowering Strategies for Disease Modification in Huntington’s Disease (2019) (149)
- The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington’s disease patients (2017) (145)
- Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mouse model of Huntington's disease. (2006) (144)
- Disease-related Prion Protein Forms Aggresomes in Neuronal Cells Leading to Caspase Activation and Apoptosis* (2005) (144)
- HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation. (2014) (141)
- Genetic risk factors for variant Creutzfeldt–Jakob disease: a genome-wide association study (2009) (141)
- Nomenclature of genetic movement disorders: Recommendations of the international Parkinson and movement disorder society task force (2016) (135)
- Rapid cell-surface prion protein conversion revealed using a novel cell system (2011) (133)
- Early changes in white matter pathways of the sensorimotor cortex in premanifest Huntington's disease (2012) (131)
- Imaging microglial activation in Huntington's disease (2007) (131)
- Mutant huntingtin impairs immune cell migration in Huntington disease. (2012) (130)
- Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression. (2012) (130)
- Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease (2012) (129)
- An exploratory double-blind, randomized clinical trial with selisistat, a SirT1 inhibitor, in patients with Huntington's disease. (2015) (127)
- Targets for future clinical trials in Huntington's disease: What's in the pipeline? (2014) (127)
- Mutant huntingtin causes defective actin remodeling during stress: defining a new role for transglutaminase 2 in neurodegenerative disease (2011) (126)
- Analysis of potential transcriptomic biomarkers for Huntington's disease in peripheral blood (2007) (125)
- The cognitive burden in Huntington's disease: Pathology, phenotype, and mechanisms of compensation (2014) (124)
- Early atrophy of pallidum and accumbens nucleus in Huntington’s disease (2010) (123)
- Treating the whole body in Huntington's disease (2015) (122)
- Nomenclature of genetic movement disorders: Recommendations of the International Parkinson and Movement Disorder Society task force (2017) (122)
- A potent and selective Sirtuin 1 inhibitor alleviates pathology in multiple animal and cell models of Huntington's disease. (2014) (121)
- Clinical impairment in premanifest and early Huntington's disease is associated with regionally specific atrophy (2011) (120)
- The ubiquitin-proteasome system in neurodegeneration. (2014) (119)
- Plasma 24S-hydroxycholesterol and caudate MRI in pre-manifest and early Huntington's disease. (2008) (116)
- Compensation in Preclinical Huntington's Disease: Evidence From the Track-On HD Study (2015) (116)
- Brain-Derived Neurotrophic Factor in Patients with Huntington's Disease (2011) (116)
- Huntington's disease phenocopies are clinically and genetically heterogeneous (2008) (115)
- The progression of regional atrophy in premanifest and early Huntington's disease: a longitudinal voxel-based morphometry study (2009) (114)
- Cannabinoid Receptor 2 Signaling in Peripheral Immune Cells Modulates Disease Onset and Severity in Mouse Models of Huntington's Disease (2012) (113)
- Increased central microglial activation associated with peripheral cytokine levels in premanifest Huntington's disease gene carriers (2015) (109)
- Metabolic characterization of the R6/2 transgenic mouse model of Huntington's disease by high-resolution MAS 1H NMR spectroscopy. (2006) (107)
- Huntington's disease: clinical presentation and treatment. (2011) (107)
- Abnormal Motor Cortex Excitability in Preclinical and Very Early Huntington's Disease (2009) (106)
- Functional compensation of motor function in pre-symptomatic Huntington's disease (2009) (103)
- Defective emotion recognition in early HD is neuropsychologically and anatomically generic (2008) (102)
- Abnormal peripheral chemokine profile in Huntington’s disease (2011) (102)
- Misfolded PrP impairs the UPS by interaction with the 20S proteasome and inhibition of substrate entry (2011) (99)
- Emotion recognition in Huntington's disease: A systematic review (2012) (99)
- High-dose creatine therapy for Huntington disease: A 2-year clinical and MRS study (2005) (99)
- Somatic and germline mosaicism in sporadic early-onset Alzheimer's disease. (2004) (98)
- Models of Parkinson's disease (2003) (97)
- Abnormal motor cortex plasticity in premanifest and very early manifest Huntington disease (2009) (94)
- Expression of mutant alpha-synuclein causes increased susceptibility to dopamine toxicity. (2000) (92)
- Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation. (2007) (91)
- A SNP in the HTT promoter alters NF-κB binding and is a bidirectional genetic modifier of Huntington disease (2015) (90)
- Altered brain mechanisms of emotion processing in pre-manifest Huntington's disease (2012) (90)
- Increased thirst and drinking in Huntington's disease and the R6/2 mouse (2008) (89)
- The structural correlates of functional deficits in early huntington's disease (2013) (89)
- KEAP1-modifying small molecule reveals muted NRF2 signaling responses in neural stem cells from Huntington's disease patients (2017) (85)
- Huntington's disease phenocopy syndromes (2007) (84)
- Clinical Features of Huntington's Disease. (2018) (83)
- A genetic association study of glutamine-encoding DNA sequence structures, somatic CAG expansion, and DNA repair gene variants, with Huntington disease clinical outcomes (2019) (83)
- Selective vulnerability of Rich Club brain regions is an organizational principle of structural connectivity loss in Huntington’s disease (2015) (83)
- Altered PDE10A expression detectable early before symptomatic onset in Huntington's disease. (2015) (82)
- MSH3 modifies somatic instability and disease severity in Huntington’s and myotonic dystrophy type 1 (2019) (82)
- Structural MRI in Huntington's disease and recommendations for its potential use in clinical trials (2013) (82)
- Biological and clinical characteristics of gene carriers far from predicted onset in the Huntington's disease Young Adult Study (HD-YAS): a cross-sectional analysis (2020) (81)
- Motor, cognitive, and functional declines contribute to a single progressive factor in early HD (2017) (79)
- DNA repair in the trinucleotide repeat disorders (2017) (79)
- Identification of an oculomotor biomarker of preclinical Huntington disease (2006) (78)
- FAN1 modifies Huntington’s disease progression by stabilizing the expanded HTT CAG repeat (2018) (78)
- Automatic detection of preclinical neurodegeneration (2009) (77)
- Alternative fates of newly formed PrPSc upon prion conversion on the plasma membrane (2013) (75)
- The application of NMR-based metabonomics in neurological disorders (2006) (74)
- Brain Regions Showing White Matter Loss in Huntington’s Disease Are Enriched for Synaptic and Metabolic Genes (2017) (73)
- The influence of nuclear background on the biochemical expression of 3460 Leber's hereditary optic neuropathy (1998) (72)
- Bone Marrow Transplantation Confers Modest Benefits in Mouse Models of Huntington's Disease (2012) (71)
- EFNS guidelines on the molecular diagnosis of mitochondrial disorders (2009) (70)
- Magnetic resonance imaging of Huntington's disease: preparing for clinical trials (2009) (69)
- Reliability and factor structure of the Short Problem Behaviors Assessment for Huntington's disease (PBA-s) in the TRACK-HD and REGISTRY studies. (2015) (67)
- Irritability in pre-clinical Huntington's disease (2010) (66)
- Whole‐brain atrophy as a measure of progression in premanifest and early Huntington's disease (2009) (62)
- The differential diagnosis of chorea (2007) (62)
- Normal in vivo skeletal muscle oxidative metabolism in sporadic inclusion body myositis assessed by 31P-magnetic resonance spectroscopy. (1998) (58)
- α-Synuclein expression in HEK293 cells enhances the mitochondrial sensitivity to rotenone (2003) (57)
- The structural involvement of the cingulate cortex in premanifest and early Huntington's disease (2011) (57)
- EFNS guidelines on the molecular diagnosis of neurogenetic disorders: general issues, Huntington’s disease, Parkinson’s disease and dystonias (2009) (56)
- Emotional face recognition deficits and medication effects in pre-manifest through stage-II Huntington's disease (2013) (56)
- Cortical dopamine dysfunction in symptomatic and premanifest Huntington's disease gene carriers (2010) (55)
- Prion-mediated neurodegeneration is associated with early impairment of the ubiquitin–proteasome system (2015) (54)
- Neurofilament light protein in blood predicts regional atrophy in Huntington disease (2018) (53)
- Expressed Alu repeats as a novel, reliable tool for normalization of real-time quantitative RT-PCR data (2010) (53)
- Visuomotor integration deficits precede clinical onset in Huntington's disease (2011) (53)
- Automated quantification of caudate atrophy by local registration of serial MRI: Evaluation and application in Huntington's disease (2009) (52)
- The impact of occipital lobe cortical thickness on cognitive task performance: An investigation in Huntington's Disease (2015) (52)
- White matter integrity in premanifest and early Huntington's disease is related to caudate loss and disease progression (2014) (51)
- Incidence of adult Huntington's disease in the UK: a UK-based primary care study and a systematic review (2016) (51)
- Characterisation of immune cell function in fragment and full-length Huntington's disease mouse models (2014) (51)
- Cerebrospinal fluid total tau concentration predicts clinical phenotype in Huntington's disease (2016) (50)
- Cerebrospinal Fluid Inflammatory Biomarkers Reflect Clinical Severity in Huntington’s Disease (2016) (50)
- Antisense oligonucleotides for neurodegeneration (2020) (49)
- A randomized, double-blind, placebo-controlled study of latrepirdine in patients with mild to moderate Huntington disease. (2013) (49)
- Cerebellar abnormalities in Huntington's disease: A role in motor and psychiatric impairment? (2014) (49)
- Operationalizing compensation over time in neurodegenerative disease (2017) (49)
- A longitudinal study of magnetic resonance spectroscopy Huntington's disease biomarkers (2015) (49)
- Lymphocyte contamination of laryngoscope blades – a possible vector for transmission of variant Creutzfeldt‐Jakob disease (2005) (48)
- Movement Disorder Society Task Force Viewpoint: Huntington's Disease Diagnostic Categories (2019) (48)
- RNA-Seq of Huntington’s disease patient myeloid cells reveals innate transcriptional dysregulation associated with proinflammatory pathway activation (2016) (48)
- An ITPR1 gene deletion causes spinocerebellar ataxia 15/16: A genetic, clinical and radiological description (2010) (47)
- The human motor cortex microcircuit: insights for neurodegenerative disease (2020) (47)
- Early recognition of Alzheimer's disease: what is consensual? What is controversial? What is practical? (1998) (46)
- In vivo characterization of white matter pathology in premanifest huntington's disease (2018) (45)
- Mutant huntingtin and neurofilament light have distinct longitudinal dynamics in Huntington’s disease (2020) (42)
- Harnessing Immune Alterations in Neurodegenerative Diseases (2009) (42)
- Oculomotor deficits indicate the progression of Huntington's disease. (2008) (41)
- Misfolded PrP and a novel mechanism of proteasome inhibition (2012) (41)
- Spinocerebellar ataxia type 17: Extension of phenotype with putaminal rim hyperintensity on magnetic resonance imaging (2005) (41)
- The Dementias Platform UK (DPUK) Data Portal (2019) (40)
- Huntington’s disease blood and brain show a common gene expression pattern and share an immune signature with Alzheimer’s disease (2017) (40)
- Correction of inter-scanner and within-subject variance in structural MRI based automated diagnosing (2014) (40)
- Topological length of white matter connections predicts their rate of atrophy in premanifest Huntington’s disease (2017) (40)
- Quality of life in Huntington's disease: a comparative study investigating the impact for those with pre-manifest and early manifest disease, and their partners. (2013) (40)
- Evaluation of multi-modal, multi-site neuroimaging measures in Huntington's disease: Baseline results from the PADDINGTON study☆ (2012) (40)
- MSH 3 modifies somatic instability and disease severity in Huntington ’ s and myotonic dystrophy type 1 (2019) (39)
- Task-Specific Training in Huntington Disease: A Randomized Controlled Feasibility Trial (2014) (39)
- An image‐based model of brain volume biomarker changes in Huntington's disease (2018) (39)
- Secondary abnormalities of mitochondrial DNA associated with neurodegeneration. (1999) (37)
- Prion degradation pathways: Potential for therapeutic intervention (2015) (37)
- Mouse models for neurological disease (2002) (37)
- A Computational Cognitive Biomarker for Early-Stage Huntington’s Disease (2016) (36)
- Stimulating neural plasticity with real‐time fMRI neurofeedback in Huntington's disease: A proof of concept study (2017) (36)
- Huntingtin Lowering Strategies for Disease Modification in Huntington’s Disease (2019) (35)
- Validation of a prognostic index for Huntington's disease (2017) (35)
- Prions and the proteasome. (2008) (35)
- Correction: PINK1 Is Necessary for Long Term Survival and Mitochondrial Function in Human Dopaminergic Neurons (2008) (35)
- Peripheral inflammation in neurodegeneration (2013) (35)
- Longitudinal Diffusion Tensor Imaging Shows Progressive Changes in White Matter in Huntington's Disease. (2015) (34)
- Visuospatial Processing Deficits Linked to Posterior Brain Regions in Premanifest and Early Stage Huntington’s Disease (2016) (34)
- Disruption of immune cell function by mutant huntingtin in Huntington's disease pathogenesis. (2016) (34)
- Creutzfeldt-Jakob disease in a young woman (1996) (34)
- Neuropsychiatry and White Matter Microstructure in Huntington’s Disease (2015) (33)
- Probabilistic classification learning with corrective feedback is selectively impaired in early Huntington’s disease—Evidence for the role of the striatum in learning with feedback (2012) (33)
- Predictors for a dementia gene mutation based on gene-panel next-generation sequencing of a large dementia referral series (2018) (32)
- Clinical Aspects of Huntington's Disease. (2015) (32)
- Gene suppression approaches to neurodegeneration (2017) (31)
- White matter predicts functional connectivity in premanifest Huntington's disease (2017) (31)
- Selective executive dysfunction but intact risky decision‐making in early Huntington's disease (2013) (31)
- Measuring compensation in neurodegeneration using MRI (2017) (31)
- Basal ganglia‐cortical structural connectivity in Huntington's disease (2015) (31)
- Loss of extra-striatal phosphodiesterase 10A expression in early premanifest Huntington's disease gene carriers (2016) (31)
- Association of CAG Repeats With Long-term Progression in Huntington Disease. (2019) (30)
- George Huntington: a legacy of inquiry, empathy and hope (2016) (30)
- Huntington disease. (2018) (30)
- G209A mutant alpha synuclein expression specifically enhances dopamine induced oxidative damage (2004) (30)
- Laquinimod dampens hyperactive cytokine production in Huntington's disease patient myeloid cells (2016) (30)
- Fluid and imaging biomarkers for Huntington's disease (2019) (29)
- Corpus callosal atrophy in premanifest and early Huntington's disease. (2013) (29)
- Recommendations for the Use of Automated Gray Matter Segmentation Tools: Evidence from Huntington’s Disease (2017) (29)
- Overlap between age-at-onset and disease-progression determinants in Huntington disease (2018) (28)
- Evaluating multicenter DTI data in Huntington's disease on site specific effects: An ex post facto approach☆ (2013) (27)
- Abnormal explicit but normal implicit sequence learning in premanifest and early Huntington's disease (2010) (27)
- Structural and functional brain network correlates of depressive symptoms in premanifest Huntington's disease (2017) (25)
- Juvenile Huntington's disease: a population-based study using the General Practice Research Database (2013) (25)
- Interregional compensatory mechanisms of motor functioning in progressing preclinical neurodegeneration (2013) (25)
- 8OHdG is not a biomarker for Huntington disease state or progression (2013) (25)
- NMDA receptor gene variations as modifiers in Huntington disease: a replication study (2011) (24)
- Testing a longitudinal compensation model in premanifest Huntington’s disease (2018) (24)
- Subcellular Localization And Formation Of Huntingtin Aggregates Correlates With Symptom Onset And Progression In A Huntington’S Disease Model (2020) (24)
- Effects of IONIS-HTTRx in Patients with Early Huntington’s Disease, Results of the First HTT-Lowering Drug Trial (CT.002) (2018) (24)
- NMDA receptor gene variations as modifiers in Huntington disease: a replication study (2011) (24)
- A critical evaluation of inflammatory markers in Huntington's Disease plasma. (2013) (23)
- Biomarker development for Huntington's disease. (2014) (23)
- Learning and recall in subjects at genetic risk for Alzheimer's disease. (2002) (22)
- Potential disease-modifying therapies for Huntington's disease: lessons learned and future opportunities (2022) (22)
- Longitudinal changes in functional connectivity of cortico‐basal ganglia networks in manifests and premanifest huntington's disease (2016) (22)
- Short-interval observational data to inform clinical trial design in Huntington's disease (2015) (21)
- Mouse models as a tool for understanding neurodegenerative diseases (2003) (21)
- Allele-Selective Suppression of Mutant Huntingtin in Primary Human Blood Cells (2017) (21)
- Stability effects on results of diffusion tensor imaging analysis by reduction of the number of gradient directions due to motion artifacts: an application to presymptomatic Huntington’s disease (2011) (21)
- Cross-sectional and longitudinal voxel-based grey matter asymmetries in Huntington's disease (2017) (21)
- Predict-HD and the future of therapeutic trials (2006) (20)
- Rate and acceleration of whole‐brain atrophy in premanifest and early Huntington's disease (2010) (20)
- Skeletal muscle atrophy in R6/2 mice - altered circulating skeletal muscle markers and gene expression profile changes. (2014) (20)
- Stability of white matter changes related to Huntington’s disease in the presence of imaging noise: a DTI study (2011) (20)
- Genetic testing in dementia — utility and clinical strategies (2020) (20)
- Sporadic inclusion body myositis not linked to prion protein codon 129 methionine homozygosity (2000) (19)
- Development of an ELISA assay for the quantification of soluble huntingtin in human blood cells (2013) (19)
- Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington’s disease mice (2017) (19)
- Structural imaging in premanifest and manifest Huntington disease. (2017) (18)
- Correction: PINK1 Is Necessary for Long Term Survival and Mitochondrial Function in Human Dopaminergic Neurons (2008) (18)
- Mitochondrial DNA in focal dystonia: A cybrid analysis (1998) (18)
- Immune markers for Huntington’s disease? (2008) (18)
- Plasma neurofilament heavy chain levels in Huntington's disease (2007) (18)
- Motor cortex synchronization influences the rhythm of motor performance in premanifest huntington's disease (2018) (17)
- Apathy predicts rate of cognitive decline over 24 months in premanifest Huntington's disease (2020) (17)
- Huntington’s Disease (2014) (17)
- Altered PDE 10 A expression detectable early before symptomatic onset in Huntington ’ s disease (2015) (17)
- Predicting clinical diagnosis in Huntington's disease: An imaging polymarker (2018) (17)
- A biological classification of Huntington's disease: the Integrated Staging System (2022) (17)
- Inconsistent emotion recognition deficits across stimulus modalities in Huntington׳s disease (2014) (17)
- Natural variation in sensory‐motor white matter organization influences manifestations of Huntington's disease (2016) (16)
- Expression of mutant exon 1 huntingtin fragments in human neural stem cells and neurons causes inclusion formation and mitochondrial dysfunction (2020) (16)
- FAN1 controls mismatch repair complex assembly via MLH1 retention to stabilize CAG repeat expansion in Huntington’s disease (2021) (16)
- Natural biological variation of white matter microstructure is accentuated in Huntington's disease (2018) (15)
- Expanding the Spectrum of Movement Disorders Associated With C9orf72 Hexanucleotide Expansions (2021) (15)
- Saccadometry of Conditional Rules in Presymptomatic Huntington's Disease (2009) (15)
- Human Huntington’s disease pluripotent stem cell-derived microglia develop normally but are abnormally hyper-reactive and release elevated levels of reactive oxygen species (2021) (15)
- Visual Working Memory Impairment in Premanifest Gene-Carriers and Early Huntington's Disease. (2012) (15)
- Premanifest and early Huntington's disease (2014) (15)
- Robust Markers and Sample Sizes for Multicenter Trials of Huntington Disease (2020) (14)
- Large‐scale brain network abnormalities in Huntington's disease revealed by structural covariance (2016) (14)
- Executive Impairment Is Associated with Unawareness of Neuropsychiatric Symptoms in Premanifest and Early Huntington’s Disease (2018) (14)
- Fronto-striatal circuits for cognitive flexibility in far from onset Huntington’s disease: evidence from the Young Adult Study (2020) (14)
- Biomarkers for Huntington's disease. (2008) (14)
- Disease Onset in Huntington's Disease: When Is the Conversion? (2021) (14)
- Grand Rounds-Hammersmith Hospital: Nocardia pericarditis (1994) (13)
- Relating quantitative 7T MRI across cortical depths to cytoarchitectonics, gene expression and connectomics (2020) (13)
- Quantification of huntingtin protein species in Huntington’s disease patient leukocytes using optimised electrochemiluminescence immunoassays (2017) (13)
- Therapeutic Antisense Targeting of Huntingtin. (2019) (13)
- Improved Diagnosis of Rare Disease Patients through Systematic Detection of Runs of Homozygosity (2020) (13)
- Corrigendum to “Increased central microglial activation associated with peripheral cytokine levels in premanifest Huntington's disease gene carriers” [Neurobiol. Dis. 83 (2015) 115–121] (2017) (13)
- Survival End Points for Huntington Disease Trials Prior to a Motor Diagnosis (2017) (12)
- Analysis of White Adipose Tissue Gene Expression Reveals CREB1 Pathway Altered in Huntington's Disease. (2015) (12)
- Reduction of confounding effects with voxel-wise Gaussian process regression in structural MRI (2014) (12)
- Mutant Huntingtin Does Not Affect the Intrinsic Phenotype of Human Huntington’s Disease T Lymphocytes (2015) (12)
- Defining pediatric huntington disease: Time to abandon the term Juvenile Huntington Disease? (2019) (12)
- Ethical issues in human prion diseases. (2003) (12)
- Biomarkers for Huntington's disease: an update. (2012) (12)
- In vivo neutralization of the protagonist role of macrophages during the chronic inflammatory stage of Huntington’s disease (2018) (11)
- Apathy and atrophy of subcortical brain structures in Huntington's disease: A two-year follow-up study (2018) (11)
- Combined cerebral atrophy score in Huntington's disease based on atlas-based MRI volumetry: Sample size calculations for clinical trials. (2019) (11)
- Inhibition of tumour necrosis factor alpha in the R6/2 mouse model of Huntington’s disease by etanercept treatment (2019) (11)
- Polyglutamine diseases (2021) (11)
- Altered Intracortical T1-Weighted/T2-Weighted Ratio Signal in Huntington’s Disease (2018) (11)
- Altered iron and myelin in premanifest Huntington's Disease more than 20 years before clinical onset: Evidence from the cross-sectional HD Young Adult Study (2021) (11)
- Impact of the control for corrupted diffusion tensor imaging data in comparisons at the group level: an application in Huntington disease (2014) (10)
- Safety and Tolerability of Selisistat for the Treatment of Huntington’s Disease: Results from a Randomized, Double-Blind, Placebo-Controlled Phase II Trial (S47.004) (2014) (10)
- Age of onset in Huntington's disease is influenced by CAG repeat variations in other polyglutamine disease-associated genes. (2017) (10)
- Test–Retest Reliability of Measures Commonly Used to Measure Striatal Dysfunction across Multiple Testing Sessions: A Longitudinal Study (2018) (10)
- Observing Huntingtons Disease: the European Huntingtons Disease Networks REGISTRY (2010) (10)
- Activity or connectivity? A randomized controlled feasibility study evaluating neurofeedback training in Huntington’s disease (2020) (10)
- Observing Huntingtons Disease: the European Huntingtons Disease Networks REGISTRY (2010) (10)
- A17 HD brain-train: neuroplasticity as a target to improve function in huntington’s disease (2016) (9)
- Diffusion imaging in Huntington’s disease: comprehensive review (2020) (9)
- Characterizing White Matter in Huntington's Disease (2019) (9)
- Increased levels of hemoglobin and alpha1-microglobulin in Huntington's disease. (2012) (9)
- An MDS Evidence‐Based Review on Treatments for Huntington's Disease (2021) (9)
- Idiopathic intimal hyperplasia of small arteries and arterioles affecting intestines and myocardium. (2003) (8)
- Working Memory-Related Effective Connectivity in Huntington’s Disease Patients (2018) (8)
- One decade ago, one decade ahead in huntington's disease (2019) (8)
- Design optimization for clinical trials in early‐stage manifest Huntington's disease (2017) (8)
- Longitudinal dynamics of mutant huntingtin and neurofilament light in Huntington's disease: the prospective HD-CSF study (2020) (8)
- F23 Validity, reliability, ability to detect change and meaningful within-patient change of the CUHDRS (2018) (8)
- The potential of composite cognitive scores for tracking progression in Huntington's disease. (2012) (8)
- Wild-type huntingtin regulates human macrophage function (2020) (8)
- Detection of Motor Changes in Huntington's Disease Using Dynamic Causal Modeling (2015) (8)
- Discovery and Early Clinical Development of ISIS-HTTRx, the First HTT-Lowering Drug to Be Tested in Patients with Huntington’s Disease (PL01.002) (2016) (7)
- Prion diseases (2011) (7)
- Reference genes selection for transcriptional profiling in blood of HD patients and R6/2 mice. (2013) (7)
- Imbalanced basal ganglia connectivity is associated with motor deficits and apathy in Huntington’s disease (2021) (6)
- Huntington’s Disease Look-alikes (2013) (6)
- Composite UHDRS Correlates With Progression of Imaging Biomarkers in Huntington's Disease (2021) (6)
- Revealing the Timeline of Structural MRI Changes in Premanifest to Manifest Huntington Disease (2021) (6)
- Patients with Huntington's disease pioneered human stereotactic neurosurgery 70 years ago (2017) (6)
- REM sleep disturbances in huntington disease (2007) (6)
- Stability and sensitivity of structural connectomes: effect of thresholding and filtering and demonstration in neurodegeneration (2018) (6)
- Medication Use in Early-HD Participants in Track-HD: an Investigation of its Effects on Clinical Performance (2016) (6)
- J01 Effects of IONIS-HTTRX (RG6042) in patients with early huntington’s disease, results of the first htt-lowering drug trial (2018) (6)
- Learning Subject-Specific Directed Acyclic Graphs With Mixed Effects Structural Equation Models From Observational Data (2018) (6)
- Apathy Associated With Impaired Recognition of Happy Facial Expressions in Huntington’s Disease (2018) (5)
- Inflammatory markers in Huntington's disease plasma—A robust nanoLC–MRM-MS assay development (2014) (5)
- D22 Compensation in preclinical huntington’s disease: evidence from the track-on HD study (2016) (5)
- Huntington's disease clinical trials corner: April 2022. (2022) (5)
- Huntingtin lowering reduces somatic instability at CAG-expanded loci (2020) (5)
- Altered Intracortical T 1 -Weighted/T 2 -Weighted Ratio Signal in Huntington’s Disease (2018) (5)
- Targeting Huntingtin in Patients with Huntington's Disease. Reply. (2019) (5)
- F59 Huntington’s disease young adult study (HD-YAS) (2018) (4)
- QUANTIFYING MUTANT HUNTINGTIN IN HUNTINGTON'S DISEASE CSF (2014) (4)
- Study of personality disorders in AIDS patients with high risk behaviors (2010) (4)
- Huntington's Disease Integrated Staging System (HD-ISS): A Novel Evidence-Based Classification System For Staging (2021) (4)
- Activity-is better than connectivity-neurofeedback training in Huntington’s disease (2018) (4)
- Therapeutic strategies for Huntington's disease. (2020) (4)
- Mislocalization of Nucleocytoplasmic Transport Proteins in Human Huntington’s Disease PSC-Derived Striatal Neurons (2021) (4)
- Longitudinal Structural MRI in Neurologically Healthy Adults (2020) (3)
- High dose creatine therapy for Huntington's disease: Clinical and 31 phosphorous magnetic resonance spectroscopy ((31P) MRS) findings in a 2-year study (2004) (3)
- PAW32 ITPR1 gene deletion causes spinocerebellar ataxia 15/16: a genetic, clinical and radiological description of a novel kindred (2010) (3)
- Timing of selective basal ganglia white matter loss in premanifest Huntington’s disease (2022) (3)
- Longitudinal expression changes are weak correlates of disease progression in Huntington’s disease (2020) (3)
- Treating Huntington’s disease (2019) (3)
- Mitochondrial abnormalities in neurodegenerative disorders (2002) (3)
- A new family with GLRB-related hyperekplexia showing chorea in homo- and heterozygous variant carriers. (2020) (3)
- A small molecule kicks repeat expansion into reverse (2020) (3)
- expansions are the most common genetic cause of Huntington ’ s disease phenocopies (2017) (2)
- Magnetic Resonance Imaging in Huntington's Disease. (2018) (2)
- A safety, tolerability and biomarker update from an ongoing open-label extension study of RG6042 in adults with early manifest Huntington’s disease (2019) (2)
- Aberrant Striatal Value Representation in Huntington's Disease Gene Carriers 25 Years Before Onset (2021) (2)
- Tracking Huntingtonʼs Disease Progression Using Motor, Functional, Cognitive, and Imaging Markers (2021) (2)
- PREVALENT MUTATIONS OF BETA- GLUBIN GENE AT THE CENTRAL PART OF EAST-AZERBAIJAN, IRAN (2004) (2)
- Reply: The repeat variant in MSH3 is not a genetic modifier for spinocerebellar ataxia type 3 and Friedreich's ataxia. (2020) (2)
- Timing of selective basal ganglia white matter loss in Huntington’s disease (2021) (2)
- J05 Legato-hd study: a phase 2 study assessing the efficacy and safety of laquinimod as a treatment for huntington disease (2018) (2)
- Stability effects on results of diffusion tensor imaging analysis by reduction of the number of gradient directions due to motion artifacts: an application to presymptomatic Huntington's disease. (2011) (2)
- The application of NMR-based metabonomics in neurological disorders (2006) (2)
- Huntington's Disease Clinical Trials Corner: November 2022. (2022) (1)
- Impact of the control for corrupted diffusion tensor imaging data in comparisons at the group level: an application in Huntington disease (2014) (1)
- Nuclear somatic mosaicism in sporadic early-onset Alzheimer's disease (2004) (1)
- H06 Cross sectional and longitudinal 3T magnetic resonance spectroscopy in a TRACK-HD cohort of individuals with premanifest and early Huntington's disease (2010) (1)
- J12 HD brain-train: enhancing neural plasticity using real-time FMRI neurofeedback training (2018) (1)
- POMD07 Quantitative assessment of biological and clinical manifestations of Huntington's disease before and after diagnosis—the TRACK-HD study (2010) (1)
- Neurofilament light-associated connectivity in young-adult Huntington’s disease is related to neuronal genes (2022) (1)
- Other genetic causes of cognitive impairment (2016) (1)
- Reply letter to Jinnah “Locus pocus” and Albanese “Complex dystonia is not a category in the new 2013 consensus classification”: Necessary evolution, no magic! (2016) (1)
- Using functional status to aid interpretation of the composite unified huntington’s disease rating scale (cUHDRS) scores in patients with huntington’s disease (HD) (2019) (1)
- Altered nuclear architecture in blood cells from Huntington’s disease patients (2021) (1)
- Survival Endpoints in Premanifest Huntington's Disease Trials: 7 Years of TRACK (2018) (1)
- A Newly Recognized HD- Phenocopy Associated with C9orf72 Expansion (2017) (1)
- CROSS SECTIONAL AND LONGITUDINAL 3T MAGNETIC RESONANCE SPECTROSCOPY IN A TRACK-HD COHORT OF INDIVIDUALS WITH PREMANIFEST AND EARLY HUNTINGTON'S DISEASE (2010) (1)
- H16 Advance care planning in huntington’s disease from the perspective of the multidisciplinary team (2018) (1)
- Mechanisms involved in the pathogenesis of Huntington's disease (1997) (1)
- Intellectual enrichment and genetic modifiers of cognition and brain volume in Huntington’s disease (2022) (1)
- Poster 9: The Short Version of the Problem Behaviours Assessment for HD (PBA-s): An Item Response Analysis Using Data from the TRACK-HD Study (2010) (1)
- Rationale and Design for LEGATO-HD Study: A Multinational, Randomized, Double-blind, Placebo-controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Laquinimod (0.5, 1.0, and 1.5 mg/day) as Treatment in Patients with Huntington Disease. (2016) (1)
- Faculty Opinions recommendation of Taming Human Genetic Variability: Transcriptomic Meta-Analysis Guides the Experimental Design and Interpretation of iPSC-Based Disease Modeling. (2018) (1)
- A2 TRACK-HD and TRACK-ON HD: yielding new insights in Huntington's disease (2012) (1)
- P3-377: A novel pathogenic pathway of immune activation detectable before cinical onset in Huntington's disease (2008) (1)
- F46 Relationship between distinct motor symptoms and apathy in huntington’s disease: clues to mechanism (2018) (1)
- A man with deteriorating ability to live independently (2011) (1)
- Abnormal molecular signatures of inflammation, energy metabolism, and vesicle biology in human Huntington disease peripheral tissues (2022) (1)
- Huntington's disease: fighting on many fronts. (2012) (1)
- Chapter 5 Mitochondrial Abnormalities in Neurodegenerative Disorders (2002) (1)
- Creatine therapy for Huntington's disease (HD): Clinical and-(31)phosphorous magnetic resonance spectroscopy (P-31 MRS) findings in a one year pilot (2002) (1)
- Opportunity cost determines action initiation latency and predicts apathy (2020) (1)
- Genetic causes of dementia (2007) (1)
- Huntington disease (2009) (1)
- CAG Somatic Instability in a Huntington Disease Expansion Carrier Presenting with a Progressive Supranuclear Palsy‐like Phenotype (2022) (1)
- Reply to ‘Topographical layer imaging as a tool to track neurodegenerative disease spread in M1’ (2020) (1)
- Validating Automated Segmentation Tools in the Assessment of Caudate Atrophy in Huntington’s Disease (2021) (1)
- Identification of an oculomotor biomarker of presymptomatic Huntington's disease (2005) (1)
- Visuospatial Processing De fi cits Linked to Posterior Brain Regions in Premanifest and Early Stage Huntington ’ s Disease (2017) (1)
- F24 Differences in companion and subject ratings of subjects' behaviour using the frontal systems behaviour scale (FrSBe)- findings from the track-hd study (2010) (1)
- Isolation of Flavobacterium branchiophilum from Rainbow Trout (Oncorhyncus mykiss) with Bacterial Gill Disease in Tabriz, Iran (2015) (1)
- DORSAL COLUMN Grey Matter Operationalizing compensation over time in neurodegenerative disease (0)
- E13 Predicting Huntington’s disease state using structural MRI: it’s more than just the striatum (2022) (0)
- A Multi-Study Model-Based Evaluation of the Sequence of Imaging and Clinical Biomarker Changes in Huntington’s Disease (2021) (0)
- F11 Applying the HD integrated staging system to the HD young adult study cohort (2022) (0)
- 241 Intrathecal antisense oligonucleotide delivery in HD: experience from RG6042 programme and best practice considerations (2022) (0)
- F02 Modelling huntington’s disease progression: interpretation, staging and prognosis (2021) (0)
- Detecting preclinical neurodegeneration: An example from Huntington's disease (2009) (0)
- Protein aggregation, the ubiquitin-proteasome system and neurodegenerative diseases (2005) (0)
- MOTORNEURON DISEASE: VEGF treatment of ALS (2005) (0)
- Dementia and neurodegeneration : Challenges and opportunities Fundamental research : From molecules to neural circuitry (2013) (0)
- A randomized, double-blind, placebo-controlled phase Ib study with selisistat (SEN0014196) in patients with Huntington's disease (2013) (0)
- Huntington's Disease and Other Choreas (2017) (0)
- F19 Exploring the issue of swallowing in huntington's disease (2010) (0)
- “On Chorea”: 150 Years of the Beginning of Hope (2022) (0)
- Automatic, inhibitory and high-order cognitive dysfunction in premanifest and early-stage Huntington's disease - the novel Say-Edgar task (2009) (0)
- Study of Lactobacillus in Cat fish (Silurus glanis) Intestine in Aras River by PCR (2012) (0)
- K4 The cost and value of a huntington’s disease multidisciplinary team meeting (2016) (0)
- Molecular Diagnosis of Ataxias and Spastic Paraplegias (2011) (0)
- D10 Neurofilament light protein in blood predicts regional atrophy in huntington’s disease (2018) (0)
- Lumbar puncture safety and tolerability in premanifest and manifest Huntington’s disease: a multi-analysis cross-sectional study (2022) (0)
- E02 Standardising and observing atrophy and cognitive patterns across the lifetime of Huntington’s disease using data from the HD-YAS and TRACK-HD and TrackOn-HD studies (2022) (0)
- Increased levels of hemoglobin and α 1-microglobulin in Huntington ’ s disease (2011) (0)
- I01 Msh3-targeting antisense oligonucleotides halt CAG repeat expansions in Huntington’s disease IPSC-derived neurons (2022) (0)
- Huntington’s disease and HD-like disorders (2014) (0)
- Machine learning spots the time to treat Huntington disease (2021) (0)
- E09 Quantitative MRI profiles across motor cortex cortical layers in premanifest Huntington’s disease using 7T MRI at 600μm resolution (2022) (0)
- B48 DNA repair pathways as a common genetic mechanism modulating the age at onset in polyglutamine diseases (2016) (0)
- Peripheral inflammation in neurodegeneration (2013) (0)
- B17 Blood transcriptome replicates dysregulation found in human huntington’s disease brain and shares an immune signature with alzheimer’s disease (2016) (0)
- C12 HTT repeat instability in family trios in the 100,000 genomes project (2022) (0)
- Poster 8: Reliability of the Problem Behaviours Assessment for HD (short version)–Data From the TRACK-HD Study (2010) (0)
- A17 Myeloid cell function in mouse models of Huntington's disease (2010) (0)
- For peer review only Juvenile Huntington's disease: an epidemiological study based on the Clinical Practice Research Datalink (2012) (0)
- Observing Huntington's Disease: the European Huntington's Disease Network's REGISTRY. (2010) (0)
- E01 Modelling the trajectory of cortical atrophy in huntington’s disease (2018) (0)
- E12 In vivo investigation of perivascular spaces in Huntington’s disease using T2-W 3T MRI (2022) (0)
- DNA REPAIR PATHWAYS MODULATE ONSET IN POLYGLUTAMINE DISEASES (2016) (0)
- A18 Cellular signalling of human monocytes in Huntington's disease (2010) (0)
- C02 FAN1 controls cag repeat expansion in huntington’s disease by dual functions, MLH1 retention and nuclease activity (2021) (0)
- D17 Extracellular vesicles biology in huntington disease (2022) (0)
- C05 The role of FAN1 nuclease activity and mlh1 binding in stabilisation of the cag repeat in HD-IPSC derived models (2022) (0)
- J09 When do voice changes affect quality of life in Huntington's disease? (2010) (0)
- H21 Outcomes of patient experience survey on video consultation via circuit at the Huntington’s disease multi-disciplinary clinic in the UK (2022) (0)
- I08 fMRI shows an distributed extrastriatal network of altered neural activation in premanifest Huntington's disease gene carriers (2010) (0)
- Diagnostic de maladies neurodégénératives (2005) (0)
- Short-interval observational data to inform clinical trial design in early Huntington ’ s Disease (2015) (0)
- Neurogenetics: Huntington's disease (2012) (0)
- E11 Compensation in huntington’s disease (2018) (0)
- C07 A CRISPRI platform to assess the role of HD risk modifiers in CAG repeat expansion in iPSC derived striatal neurons (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)
- B14 Development of a high content imaging platform for hd therapeutic screening using human medium spiny neurons derived from an hd family ipsc series (2018) (0)
- Faculty Opinions recommendation of Reproducibility of Molecular Phenotypes after Long-Term Differentiation to Human iPSC-Derived Neurons: A Multi-Site Omics Study. (2018) (0)
- B24 Assessment of immune system activation status during the course of disease in huntington’s disease mouse model (2016) (0)
- D18 Brain network breakdown and pathophysiological correlates in huntington’s disease (2016) (0)
- ACNR MJ07:Layout 1 (2007) (0)
- In vivo neutralization of the protagonist role of macrophages during the chronic inflammatory stage of Huntington’s disease (2018) (0)
- Awareness in Huntington’s Disease Manuscript 1 Executive impairment is associated with unawareness of neuropsychiatric symptoms in premanifest and early Huntington’s disease (2018) (0)
- The limbic-circuitry of Huntington's disease: A combined neuroimaging approach (2009) (0)
- G1 Executive task performance and anxiety are associated with self-awareness of neuropsychiatric symptoms in huntington’s disease (2016) (0)
- Response to the letter to the editor by Reilmann et al referring to our article titled “Motor cortex synchronization influences the rhythm of motor performance in premanifest Huntington's disease” (2018) (0)
- F13 Safety and tolerability of lumbar punctures (LP) procedure in patients with huntington’s disease (2021) (0)
- Investigation of PINK1 dysfunction in Parkinson's disease (2006) (0)
- D4 Prediction of huntington’s disease phenotype by cerebrospinal fluid biomarkers of inflammation and cell death (2016) (0)
- Automated Segmentation of Cortical Grey Matter from T1-Weighted MRI Images. (2019) (0)
- Large C9ORF72 hexanucleotide expansions arise spontaneously in the healthy population but can be distinguished from pathogenic mutations by Sothern Blotting (2012) (0)
- Abnormal molecular signatures of inflammation, energy metabolism, and vesicle biology in human Huntington disease peripheral tissues (2022) (0)
- Monitoring huntintong disease. (2005) (0)
- B10 Inclusion formation in mutant HTT exon 1 expressing human neuronal cells (2016) (0)
- EXPLORING THE ISSUE OF SWALLOWING IN HUNTINGTON'S DISEASE (2010) (0)
- Reply to ‘Topographical layer imaging as a tool to track neurodegenerative disease spread in M1’ (2020) (0)
- E03 Uncovering the temporal sequence of regional brain volume and neural connectivity changes in huntington’s disease (2021) (0)
- B13 Huntington’s disease phenotypes and disrupted corticostriatal connectivity observed in a novel ipsc-derived in vitro co-culture model (2018) (0)
- Brain phosphodiesterase 10A (PDE-10A) density in early premanifest HD gene carriers (2014) (0)
- Learning Directed Acyclic Graphs with Mixed Effects Structural Equation Models from Observational Data (2018) (0)
- Task-Specific Training in Huntington Disease: A Randomized Controlled Feasibility Trial (2014) (0)
- A small molecule kicks repeat expansion into reverse (2020) (0)
- D20 Operationalising compensation over time in neurodegenerative disease (2016) (0)
- D21 Longitudinal compensation in the cognitive network in huntington’s disease (2016) (0)
- P3-375 Correlation between patterns of atrophy and psychomotor task performance in early huntington's disease (2004) (0)
- The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington’s disease patients (2017) (0)
- B49 Genetic modifiers of huntington’s disease progression (2016) (0)
- Applying the Huntington's Disease Integrated Staging System (HD-ISS) to Observational Studies. (2023) (0)
- Huntington's Disease for Brain Mapping: An Encyclopedic Reference (2015) (0)
- Chapter 22 – Prion diseases of humans and animals (2010) (0)
- B25 Mitochondrial fission and fusion in skeletal muscle from HD patients and zQ175 mice (2016) (0)
- Progressive alterations in white matter microstructure across the timecourse of Huntington's disease (2023) (0)
- Reduced Neurite Density in Pre-manifest Huntington’s Disease Population detected by NODDI (2015) (0)
- D19 Longitudinal changes in functional connectivity of cortico-basal ganglia networks in manifest and premanifest huntington’s disease (2016) (0)
- Genetic modifier for Huntington's disease progression identified (2020) (0)
- A PET-CT study on neuroinflammation in Huntington’s disease patients participating in a randomized trial with laquinimod (2023) (0)
- PINK1 is necessary for long term survival and mitochondrial function in primary human dopaminergic neurons (2009) (0)
- Gene suppression approaches to neurodegeneration (2017) (0)
- Current Opinion in Neurology Therapeutic strategies for Huntington’s disease (2020) (0)
- Opportunity cost determines free-operant action initiation latency and predicts apathy (2021) (0)
- Neuron NeuroView Harnessing Immune Alterations in Neurodegenerative Diseases (2009) (0)
- D08 Neurofilament light protein in blood as a potential biomarker of neurodegeneration in hungtington’s disease: a retrospective cohort analysis (2018) (0)
- J9 Probing huntington’s disease phenocopy syndromes with next-generation sequencing (2016) (0)
- Mitochondrial dysfunction in the pathogenesis of neurodegeneration. (2000) (0)
- Monitorierung of Huntington's Disease (2005) (0)
- Somatic mosaicism in sporadic early-onset Alzheimer's disease (2004) (0)
- Huntington Study Group Abstracts 2020 (2020) (0)
- D16 White matter microstructure and natural biological variation in huntington’s disease (2016) (0)
- Human Huntington’s disease pluripotent stem cell-derived microglia develop normally but are abnormally hyper-reactive and release elevated levels of reactive oxygen species (2021) (0)
- F22 Robust biomarkers of huntington’s disease progression: observations from the track-hd, predict-hd and image-hd studies (2018) (0)
- F03 Fine-grained prediction of huntington’s disease progression using a stacked ensemble approach (2021) (0)
- Title: Imbalanced basal ganglia connectivity is associated with motor deficits and apathy in Huntington’s disease (2021) (0)
- Inhibition of tumour necrosis factor alpha in the R6/2 mouse model of Huntington’s disease by etanercept treatment (2019) (0)
- 2.039 LONGITUDINAL PET STUDIES OF MICROGLIAL ACTIVATION IN HUNTINGTON'S DISEASE (2012) (0)
- F21 Cag-dependent huntington’s disease patterns over decades: the track-hd and track-on studies (2018) (0)
- Structural alterations of amygdala and hippocampus and HPA axis dysregulation in patients with borderline personality disorder (2005) (0)
- Molecular Diagnosis of Neurogenetic Disorders: General Issues, Huntington's Disease, Parkinson's Disease and Dystonias (2011) (0)
- PolyQ length-dependent metabolic alterations and DNA damage drive human astrocyte dysfunction in Huntington’s disease (2023) (0)
- P3-385 Prion infection renders neuronal cells more susceptible to proteasome inhibition and apoptotic stimuli (2004) (0)
- Editorial Board (2010) (0)
- Abnormal explicit but intact implicit sequence learning in presymptomatic and early Huntington's disease (2009) (0)
- G03 Visuomotor integration deficits in premanifest and early manifest Huntington's disease in the TRACK-HD study (2010) (0)
- B15 Innate transcriptional dysregulation is associated with proinflammatory pathway activation in huntington’s disease myeloid cells (2016) (0)
- 234 Using functional status to aid interpretation of cUHDRS scores in patients with Huntington’s disease (2022) (0)
- TABRIZI, Sarah J.: London/UK (2019) (0)
- Edinburgh Research Explorer Targeting Huntingtin Expression in Patients with Huntington’s Disease (2019) (0)
- E01 The HD young adult study 2: longitudinal follow up (2022) (0)
- Exploring the relationship between quantitative 7T MRI, cytoarchitectonics and gene expression (2019) (0)
- D8 Tms-eeg markers of inhibitory deficits in huntington’s disease (2016) (0)
- INFECTION OF THE CAT FISH (SILURUS GLANIS) WITH PROTEOCEPHALUS OSCULATUS AND SILURITAENIA SILURI IN ARAS DAM (2014) (0)
- Structural and Functional Brain Network Correlates of Depressive Symptoms in Premanifest Huntingtons Disease (2017) (0)
- A30 The role of huntingtin in human myeloid cells (2018) (0)
- G.P.377 NeurOmics: EU-funded-omics research for diagnosis and therapy in rare neuromuscular and neurodegenerative diseases (2015) (0)
- 1609 Length of white matter connexions determine their rate of atrophy in premanifest huntington’s disease (2017) (0)
- STUDY OF EFFECT SEVERAL LEVELS OF MULTI-ENZYME ON PERFORMANCE PARAMETERS AND SURVIVAL RATE IN RAINBOW TROUT (ONCORHYNCUS MYKISS) (2011) (0)
- Molecular Diagnosis of Channelopathies, Epilepsies, Migraine, Stroke and Dementias (2011) (0)
- Molecular Diagnosis of Mitochondrial Disorders (2011) (0)
- PRIMARY AND SECONDARY DEFICIENCIES OF THE MITOCHONDRIAL RESPIRATORY CHAIN (1998) (0)
- J13 Distributed neural networks associated with emotion processing are altered in premanifest Huntington's disease and correlated with genetic load (2012) (0)
- CAN AMIDE PROTON TRANSFER MRI GIVE ADDITIONAL INFORMATION ABOUT HUNTINGTON'S DISEASE? (2016) (0)
- GENETIC MODIFIERS OF HUNTINGTON'S DISEASE PROGRESSION (2016) (0)
- Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington’s disease mice (2017) (0)
- Multimodal characterization of the visual network in Huntington’s disease gene carriers (2019) (0)
- The human motor cortex microcircuit: insights for neurodegenerative disease (2020) (0)
- Biomarkers for Huntington's disease - promises and challenges (2013) (0)
- C01 Glutamine codon usage and somatic mosaicism of the HTT cag repeat are modifiers of huntington disease severity (2018) (0)
- D06 Influencing Huntington’s disease monitoring and therapeutic development with remote quantification of blood neurofilament light protein (2022) (0)
- O3-02-01 Nuclear somatic mosaicim in sporadic early-onset Alzheimer's disease (2004) (0)
- The limbic-circuitry of Huntington's disease (2009) (0)
- Disentangling the Connection Between Neurodevelopment and Neurodegeneration in Huntington's Disease (2022) (0)
- #3101 Imbalanced basal ganglia connectivity is associated with motor deficits and apathy in Huntingtons disease: first evidence from human in vivo neuroimaging (2021) (0)
- Suppression of Somatic Expansion As a Novel Therapeutic Approach for Huntington Disease and Other Repeat Expansion Disorders (2022) (0)
- Molecular Diagnosis of Neurogenetic Disorders: Motoneuron, Peripheral Nerve and Muscle Disorders (2011) (0)
- Forecasting individual progression trajectories in Huntington disease enables more powered clinical trials (2022) (0)
- An ITPR1 gene deletion causes SCA15 and 16; a genetic, clinical and radiological description (2011) (0)
- Faculty of 1000 evaluation for Faulty neuronal determination and cell polarization are reverted by modulating HD early phenotypes. (2018) (0)
- Visuomotor integration with direct and indirect visual feedback in Huntington's disease - a circle tracing task (2009) (0)
- 9 Aberrant striatal value representation in Huntington’s disease gene carriers 25 years before onset (2020) (0)
- B27 Abnormal bioenergetics in inclusion-containing mutant HTT exon 1 primary human neurons (2016) (0)
- C06 Genetic variation in MSH3 that lowers its expression ameliorates disease course and limits repeat expansion in huntington’s disease and myotonic dystrophy type 1 (2018) (0)
- F45 Apathy associated with impaired recognition of happy facial expressions in huntington’s disease (2018) (0)
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