ArticlePloS one2014
Deep white matter in Huntington's disease.
Article in PloS one, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed.
- Psychosis in Huntington disease: a short update.Journal of neural transmission (Vienna, Austria : 1996) · 2026Review
- Along-Tract Diffusion Alterations in the Dentato-Rubro-Thalamic Tract Correlate With Motor and Cognitive Decline in Huntington's Disease.Human brain mapping · 2025Article
- Neuroimaging Techniques in Huntington's Disease: A Critical Review.Movement disorders clinical practice · 2025Review
- Shared patterns of glial transcriptional dysregulation link Huntington's disease and schizophrenia.Brain : a journal of neurology · 2024Article
- Early whole-body mutant huntingtin lowering averts changes in proteins and lipids important for synapse function and white matter maintenance in the LacQ140 mouse model.Neurobiology of disease · 2023Article
- Early whole-body mutant huntingtin lowering averts changes in proteins and lipids important for synapse function and white matter maintenance in the LacQ140 mouse model.bioRxiv : the preprint server for biology · 2023Article
- Progressive alterations in white matter microstructure across the timecourse of Huntington's disease.Brain and behavior · 2023Article
- Contribution of Glial Cells to Polyglutamine Diseases: Observations from Patients and Mouse Models.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2023Review
- Mutation-related magnetization-transfer, not axon density, drives white matter differences in premanifest Huntington disease: Evidence from in vivo ultra-strong gradient MRI.Human brain mapping · 2022Article
- Molecular Pathophysiological Mechanisms in Huntington's Disease.Biomedicines · 2022Review
- Brain Alterations in Aged OVT73 Sheep Model of Huntington's Disease: An MRI Based Approach.Journal of Huntington's disease · 2022Article
- Non-Cell Autonomous and Epigenetic Mechanisms of Huntington's Disease.International journal of molecular sciences · 2021Review
- Broad white matter impairment in multiple system atrophy.Human brain mapping · 2021Article
- Fixel-Based Analysis Effectively Identifies White Matter Tract Degeneration in Huntington's Disease.Frontiers in neuroscience · 2021Article
- Diffusion imaging in Huntington's disease: comprehensive review.Journal of neurology, neurosurgery, and psychiatry · 2020Review
- A Critical Review of White Matter Changes in Huntington's Disease.Movement disorders : official journal of the Movement Disorder Society · 2020Review
- Detection of axonal degeneration in a mouse model of Huntington's disease: comparison between diffusion tensor imaging and anomalous diffusion metrics.Magma (New York, N.Y.) · 2019Article
- Myelin Breakdown in Human Huntington's Disease: Multi-Modal Evidence from Diffusion MRI and Quantitative Magnetization Transfer.Neuroscience · 2019Article
- Loss-of-Huntingtin in Medial and Lateral Ganglionic Lineages Differentially Disrupts Regional Interneuron and Projection Neuron Subtypes and Promotes Huntington's Disease-Associated Behavioral, Cellular, and Pathological Hallmarks.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2019Article
- Inhibition of Drp1 hyperactivation reduces neuropathology and behavioral deficits in zQ175 knock-in mouse model of Huntington's disease.Biochemical and biophysical research communications · 2018Article
Corrections and comments
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Authors and funding
7 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
White matter (WM) abnormalities have already been shown in presymptomatic (Pre-HD) and symptomatic HD subjects using Magnetic Resonance Imaging (MRI). In the present study, we examined the microstructure of the long-range large deep WM tracts by applying two different MRI approaches: Diffusion Tensor Imaging (DTI) -based tractography, and T2*weighted (iron sensitive) imaging. We collected Pre-HD subjects (n = 25), HD patients (n = 25) and healthy control subjects (n = 50). Results revealed increased axial (AD) and radial diffusivity (RD) and iron levels in Pre-HD subjects compared to controls. Fractional anisotropy decreased between the Pre-HD and HD phase and AD/RD increased and although impairment was pervasive in HD, degeneration occurred in a pattern in Pre-HD. Furthermore, iron levels dropped for HD patients. As increased iron levels are associated with remyelination, the data suggests that Pre-HD subjects attempt to repair damaged deep WM years before symptoms occur but this process fails with disease progression.
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Registered trials
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