Huntington's disease is a four-repeat tauopathy with tau nuclear rods
Open accessAbstract
An imbalance of tau isoforms containing either three or four microtubule-binding repeats causes frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17) in families with intronic mutations in the MAPT gene. Here we report equivalent imbalances at the mRNA and protein levels and increased total tau levels in the brains of subjects with Huntington's disease (HD) together with rod-like tau deposits along neuronal nuclei. These tau nuclear rods show an ordered filamentous ultrastructure and can be found filling the neuronal nuclear indentations previously reported in HD brains. Finally, alterations in serine/arginine-rich splicing factor-6 coincide with tau missplicing, and a role of tau in HD pathogenesis is evidenced by the attenuation of motor abnormalities of mutant HTT transgenic mice in tau knockout backgrounds.
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In HD models, tau ablation reduces motor abnormalities and restores neurite length.
“while in HD models, ablation led to reduced motor abnormalities and restoration of neurite length [83, 84].”
From Tau Ablation Rewires Brain Cell Programs in Health and Restores Function in Disease (2026) · cites this paper as [84]
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References 27 · Cited by 231
- Authors
- Fernández-Nogales, M., Cabrera, J. R., Santos-Galindo, M., Hoozemans, J. J. M., Ferrer, I., Rozemuller, A. J. M., Hernández, F., Avila, J., & Lucas, J. J.
- Affiliations
- Consejo Superior de Investigaciones Científicas (ES)
- Amsterdam Neuroscience (NL)
- Corresponding author
- Lucas, José J
- Venue
- Nature Medicine
- Published
- 2014-07-20
- Volume
- 20
- Issue
- 8
- Pages
- 881-885
- ISSN
- 1078-8956 (ISSN-L)
- 1546-170X
- OpenAlex
- W2057886219
- Cited by
- 231
- References
- 27
- Citations by year
- 2026: 14 · 2025: 5 · 2024: 19 · 2023: 18 · 2022: 33 · 2021: 17 · 2020: 24 · 2019: 16 · 2018: 13 · 2017: 23 · 2016: 28 · 2015: 15 · 2014: 5
- FWCI
- 10.82
- Type
- journal article
- Language
- en
- Open access
- green
- hdl.handle.net
- Licence
- other-oa
- www.springer.com/tdm (tdm)
- Locations
- Nature Medicine (publishedVersion)
- PubMed (publishedVersion)
- Amsterdam UMC (VU Amsterdam) - Institutional Repository (publishedVersion)
- DIGITAL.CSIC (Spanish National Research Council (CSIC)) (submittedVersion, other-oa)
- DIGITAL.CSIC (Spanish National Research Council (CSIC)) (submittedVersion)
- Pure Amsterdam UMC (submittedVersion)
- Zenodo (CERN European Organization for Nuclear Research) (submittedVersion, other-oa)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- Huntington's disease, tauopathy, tau isoforms, microtubule-binding repeats, MAPT, frontotemporal dementia with parkinsonism linked to chromosome 17, tau knockout mice, motor abnormalities, neurodegeneration
- MeSH terms
- Serine-Arginine Splicing Factors, Animals, Brain, Humans, Huntington Disease, Mice, Transgenic, Mutation, Nuclear Proteins, Phosphoproteins, RNA, Messenger, RNA-Binding Proteins, tau Proteins, Alternative Splicing, Mice, Knockout, Protein Isoforms, Tauopathies, Serotonin Plasma Membrane Transport Proteins, Mice, Frontotemporal Dementia
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