Bone Marrow Transplantation Confers Modest Benefits in Mouse Models of Huntington's Disease
Open accessAbstract
Huntington's disease (HD) is caused by an expanded polyglutamine tract in the protein huntingtin (htt). Although HD has historically been viewed as a brain-specific disease, htt is expressed ubiquitously, and recent studies indicate that mutant htt might cause changes to the immune system that could contribute to pathogenesis. Monocytes from HD patients and mouse models are hyperactive in response to stimulation, and increased levels of inflammatory cytokines and chemokines are found in pre-manifest patients that correlate with pathogenesis. In this study, wild-type (WT) bone marrow cells were transplanted into two lethally irradiated transgenic mouse models of HD that ubiquitously express full-length htt (YAC128 and BACHD mice). Bone marrow transplantation partially attenuated hypokinetic and motor deficits in HD mice. Increased levels of synapses in the cortex were found in HD mice that received bone marrow transplants. Importantly, serum levels of interleukin-6, interleukin-10, CXC chemokine ligand 1, and interferon-γ were significantly higher in HD than WT mice but were normalized in mice that received a bone marrow transplant. These results suggest that immune cell dysfunction might be an important modifier of pathogenesis in HD.
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Transplantation of bone marrow from WT mice into HD mice partially alleviates motor deficits, elevates cortical synaptic levels and reduces serum inflammatory factors including IL-6, IL-10, CXC chemokine ligand 1, and IFN-γ.
“transplantation of bone marrow from WT mice into HD mice partially alleviates motor deficits, elevates cortical synaptic levels and reduces serum inflammatory factors, including IL-6, IL-10, CXC chemokine ligand 1, and IFN-γ.458”
From Antiageing strategy for neurodegenerative diseases: from mechanisms to clinical advances (2025) · cites this paper as [458]
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Is the Immunological Response a Bottleneck for Cell Therapy in Neurodegenerative Diseases?
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Are immunotherapies for Huntington’s disease a realistic option?
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The Emerging Landscape of Natural Small-molecule Therapeutics for Huntington’s Disease
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Antibody-based therapies for Huntington’s disease: current status and future directions
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Wild-type huntingtin regulates human macrophage function
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Self-citationRETRACTED: Recent approaches in regenerative medicine in the fight against neurodegenerative disease
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Self-citation
- Authors
- Kwan, W., Magnusson, A., Chou, A., Adame, A., Carson, M. J., Kohsaka, S., Masliah, E., Möller, T., Ransohoff, R., Tabrizi, S. J., Björkqvist, M., & Muchowski, P. J.
- Affiliations
- University of California, San Francisco (US)
- Lund University (SE)
- University of California San Diego (US)
- University of California, Riverside (US)
- National Center of Neurology and Psychiatry (JP)
- University of Washington (US)
- Cleveland Clinic (US)
- UCL Queen Square Institute of Neurology (GB)
- ORCID
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 2012-01-04
- Volume
- 32
- Issue
- 1
- Pages
- 133-142
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- PMC
- PMC3571858
- OpenAlex
- W2007024808
- Cited by
- 75
- References
- 52
- Citations by year
- 2025: 1 · 2024: 3 · 2023: 6 · 2022: 4 · 2021: 8 · 2020: 7 · 2019: 2 · 2018: 3 · 2017: 7 · 2016: 6 · 2015: 8 · 2014: 10 · 2013: 3 · 2012: 7
- FWCI
- 3.69
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Neuroscience (publishedVersion, cc-by-nc-sa)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Lund University Publications (Lund University) (submittedVersion, other-oa)
- Funders
- Gladstone Institutes
- National Institutes of Health
- University of California, San Francisco
- Medical Research Council (MR/J003832/1)
- National Institute of Neurological Disorders and Stroke (P30NS065780)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Neuroscience › Neurology › Neuroinflammation and Neurodegeneration Mechanisms
- Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
- Keywords
- Huntington's disease, bone marrow transplantation, huntingtin, polyglutamine expansion, immune dysfunction, monocytes, inflammatory cytokines, chemokines, interleukin-6, IL-10, CXCL1, interferon-gamma, motor impairment, synaptogenesis, YAC128 mice, BACHD mice, neuroinflammation
- MeSH terms
- Animals, Bone Marrow Cells, Disease Models, Animal, Female, Humans, Huntington Disease, Immunosuppression Therapy, Male, Mice, Inbred C57BL, Mice, Transgenic, Bone Marrow Transplantation, Autoimmune Diseases of the Nervous System, Mice
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