ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2021
Mutant Huntingtin Is Cleared from the Brain via Active Mechanisms in Huntington Disease.
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Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 42 papers.
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42citing papers in PubMed
3.6field-weighted citation impact, top 6% of its field
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Who cites it
42 citing papers in PubMed, 65 citations in OpenAlex.
- Berbamine hydrochloride as a brain-penetrant galectin-3 inhibitor in a model of Huntington's disease.Brain : a journal of neurology · 2026Article
- Functional and Structural Evidence of Neurofluid Circuit Aberrations in Huntington Disease.Annals of clinical and translational neurology · 2026Article
- The Impact of Neurotoxin Proteins Trafficked by Primary Cilia and Extracellular Vesicles in Neurodegenerative Diseases.Biology · 2025Review
- Molecular and imaging biomarker responses to brain mutant HTT lowering in a mouse model of Huntington disease.Molecular therapy. Nucleic acids · 2025Article
- The emerging role of biomaterial applications in cerebral lymphatic surgical interventions: A narrative review.Biomaterials and biosystems · 2025Review
- Neuroinflammation in Huntington's disease: Causes, consequences, and treatment strategies.Journal of Huntington's disease · 2025Review
- The glymphatic system in Huntington's disease.Journal of Huntington's disease · 2025Review
- Diffusion along Perivascular Spaces as a Marker for Glymphatic System Impairment in Huntington's Disease.Movement disorders : official journal of the Movement Disorder Society · 2025Article
- A Practical Guide for Diagnostic Investigations and Special Considerations in Patients With Huntington's Disease in Korea.Journal of movement disorders · 2025Article
- Sleep abnormalities are associated with greater cognitive deficits and disease activity in Huntington's disease: a 12-year polysomnographic study.Brain communications · 2025Article
- Glymphatic influx and clearance are perturbed in Huntington's disease.JCI insight · 2024Article
- Elevated plasma and CSF neurofilament light chain concentrations are stabilized in response to mutant huntingtin lowering in the brains of Huntington's disease mice.Translational neurodegeneration · 2024Article
- Challenges and Future Perspectives in Modeling Neurodegenerative Diseases Using Organ-on-a-Chip Technology.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2024Review
- Exploring molecular mechanisms, therapeutic strategies, and clinical manifestations of Huntington's disease.Archives of pharmacal research · 2024Review
- Astrocytes: The Stars in Neurodegeneration?Biomolecules · 2024Review
- Current Understanding of the Anatomy, Physiology, and Magnetic Resonance Imaging of Neurofluids: Update From the 2022 "ISMRM Imaging Neurofluids Study group" Workshop in Rome.Journal of magnetic resonance imaging : JMRI · 2024Review
- A circadian clock regulates the blood-brain barrier across phylogeny.Vitamins and hormones · 2024Review
- Exploring neurodegenerative disorders using advanced magnetic resonance imaging of the glymphatic system.Frontiers in psychiatry · 2024Review
- Cerebrospinal Fluid Flow in Patients with Huntington's Disease.Annals of neurology · 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.Neurobiology of disease · 2023Article
Corrections and comments
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Authors and funding
19 authors at 8 institutions in 4 countries.
Nicholas S CaronCentre for Molecular Medicine and Therapeutics, British Columbia Children's Hospital Research Institute, Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia V5Z 4H4, Canada.
Raul BanosBurnett School of Biomedical Sciences, University of Central Florida, Orlando, Florida 32828.
Christopher YanickBurnett School of Biomedical Sciences, University of Central Florida, Orlando, Florida 32828.
Amirah E AlyCentre for Molecular Medicine and Therapeutics, British Columbia Children's Hospital Research Institute, Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia V5Z 4H4, Canada.
Lauren M ByrneUCL Huntington's Disease Centre, UCL Queen Square Institute of Neurology, University College London, London, WC1N 3BG, United Kingdom.
Ethan D SmithBurnett School of Biomedical Sciences, University of Central Florida, Orlando, Florida 32828.
Yuanyun XieBurnett School of Biomedical Sciences, University of Central Florida, Orlando, Florida 32828.
Stephen E P SmithSeattle Children's Research Institute, Center for Integrative Brain Research, Seattle, Washington 98101.
Nalini PotluriBurnett School of Biomedical Sciences, University of Central Florida, Orlando, Florida 32828.
Hailey Findlay BlackCentre for Molecular Medicine and Therapeutics, British Columbia Children's Hospital Research Institute, Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia V5Z 4H4, Canada.
Lorenzo CasalCentre for Molecular Medicine and Therapeutics, Vancouver, British Columbia V5Z 4H4, Canada.
Seunghyun KoCentre for Molecular Medicine and Therapeutics, Vancouver, British Columbia V5Z 4H4, Canada.
Daphne CheungCentre for Molecular Medicine and Therapeutics, Vancouver, British Columbia V5Z 4H4, Canada.
Hyeongju KimDepartment of Biological Sciences, KAIST, Daejeon, Korea, 34141.
Ihn Sik SeongDepartment of Neurology, Massachusetts General Hospital, Boston, Massachusetts 02114.
Edward J WildUCL Huntington's Disease Centre, UCL Queen Square Institute of Neurology, University College London, London, WC1N 3BG, United Kingdom.
Ji-Joon SongDepartment of Biological Sciences, KAIST, Daejeon, Korea, 34141.
Michael R HaydenCentre for Molecular Medicine and Therapeutics, British Columbia Children's Hospital Research Institute, Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia V5Z 4H4, Canada amber.southwell@ucf.edu mrh@cmmt.ubc.ca.
Amber L SouthwellBurnett School of Biomedical Sciences, University of Central Florida, Orlando, Florida 32828 amber.southwell@ucf.edu mrh@cmmt.ubc.ca.
University of Central Florida · USPacific Centre for Reproductive Medicine · CABC Children's Hospital · CAKorea Advanced Institute of Science and Technology · KRUniversity of British Columbia · CAMassachusetts General Hospital · USNational Hospital for Neurology and Neurosurgery · GBUniversity College London · GB
Funding
Delineating factors that modulate huntingtin secretion and its role in Huntington disease pathogenesisR01NS116099 · NINDS · UNIVERSITY OF CENTRAL FLORIDA · PI SOUTHWELL, AMBER L · 2020 to 2024
$2.1MCIHR FDN-154278Medical Research Council MR/M008592/1NINDS NIH HHS R01 NS116099
Abstract
Huntington disease (HD) is a neurodegenerative disease caused by a CAG trinucleotide repeat expansion in the huntingtin (
Indexed as
Brain ChemistryAnimalsAstrocytesBiomarkersFemaleGlymphatic SystemHumansHuntingtin ProteinHuntington DiseaseMaleMiceMice, TransgenicMutationNeuronsTrinucleotide Repeat ExpansionBiomarkersHTT protein, humanHuntingtin Proteinbiomarkercerebrospinal fluidglymphatic systemHuntington diseaseneurodegenerationprotein secretion
Identifiers
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Registered trials
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.