The DOI matches its Crossref record; 5 of the 5 references it cites were found in a registry or an index.
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease
Open accessAbstract
Huntington's disease (HD) is an inherited neurodegenerative disorder characterized by both neurological and systemic abnormalities. We examined the peripheral immune system and found widespread evidence of innate immune activation detectable in plasma throughout the course of HD. Interleukin 6 levels were increased in HD gene carriers with a mean of 16 years before the predicted onset of clinical symptoms. To our knowledge, this is the earliest plasma abnormality identified in HD. Monocytes from HD subjects expressed mutant huntingtin and were pathologically hyperactive in response to stimulation, suggesting that the mutant protein triggers a cell-autonomous immune activation. A similar pattern was seen in macrophages and microglia from HD mouse models, and the cerebrospinal fluid and striatum of HD patients exhibited abnormal immune activation, suggesting that immune dysfunction plays a role in brain pathology. Collectively, our data suggest parallel central nervous system and peripheral pathogenic pathways of immune activation in HD.
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Cites
Classification and prediction of clinical Alzheimer's diagnosis based on plasma signaling proteins
Ray, S., Britschgi, M., Herbert, C., Takeda-Uchimura, Y., Boxer, A., Blennow, K., Friedman, L. F., Galasko, D. R., Jutel, M., Karydas, A., et al. (2007) · Nature Medicine · doi:10.1038/nm1653 · Cited by 1,048
Microglial activation correlates with severity in Huntington disease: A clinical and PET study
Pavese, N., Gerhard, A., Tai, Y. F., Ho, A. K., Turkheimer, F., Barker, R. A., Brooks, D. J., & Piccini, P. (2006) · Neurology · doi:10.1212/01.wnl.0000222734.56412.17 · Cited by 462
Microglial activation in presymptomatic Huntington's disease gene carriers
Tai, Y. F., Pavese, N., Gerhard, A., Tabrizi, S. J., Barker, R. A., Brooks, D. J., & Piccini, P. (2007) · Brain · doi:10.1093/brain/awm044 · Cited by 440
Self-citationCreatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2′dG
Hersch, S. M., Gevorkian, S., Marder, K., Moskowitz, C., Feigin, A., Cox, M., Como, P., Zimmerman, C., Lin, M., Zhang, L., et al. (2006) · Neurology · doi:10.1212/01.wnl.0000194318.74946.b6 · Cited by 313
Expression and biologic characterization of the murine chemokine KC.
Bozic, C. R., Kolakowski, L. F., Gerard, N. P., Garcia-Rodriguez, C., von Uexkull-Guldenband, C., Conklyn, M. J., Breslow, R., Showell, H. J., & Gerard, C. (1995) · The Journal of Immunology · doi:10.4049/jimmunol.154.11.6048 · Cited by 294
Cited by
Huntington's disease: from molecular pathogenesis to clinical treatment
Ross, C. A., & Tabrizi, S. J. (2011) · The Lancet Neurology · doi:10.1016/s1474-4422(10)70245-3 · Cited by 1,743
Self-citationBates, G. P., Dorsey, R., Gusella, J. F., Hayden, M. R., Kay, C., Leavitt, B. R., Nance, M., Ross, C. A., Scahill, R. I., Wetzel, R., et al. (2015) · Nature Reviews Disease Primers · doi:10.1038/nrdp.2015.5 · Cited by 1,641
Self-citationInnate immune activation in neurodegenerative disease
Heneka, M. T., Kummer, M. P., & Latz, E. (2014) · Nature Reviews Immunology · doi:10.1038/nri3705 · Cited by 1,437
Huntington disease: natural history, biomarkers and prospects for therapeutics
Ross, C. A., Aylward, E. H., Wild, E. J., Langbehn, D. R., Long, J. D., Warner, J. H., Scahill, R. I., Leavitt, B. R., Stout, J. C., Paulsen, J. S., et al. (2014) · Nature Reviews Neurology · doi:10.1038/nrneurol.2014.24 · Cited by 1,016
Self-citationMicroglia-mediated neuroinflammation in neurodegenerative diseases
Subhramanyam, C. S., Wang, C., Hu, Q., & Dheen, S. T. (2019) · Seminars in Cell & Developmental Biology · doi:10.1016/j.semcdb.2019.05.004 · Cited by 870
Metadata
- Authors
- Björkqvist, M., Wild, E. J., Thiele, J., Silvestroni, A., Andre, R., Lahiri, N., Raibon, E., Lee, R. V., Benn, C. L., Soulet, D., Magnusson, A., Woodman, B., Landles, C., Pouladi, M. A., Hayden, M. R., Khalili-Shirazi, A., Lowdell, M. W., Brundin, P., Bates, G. P., Leavitt, B. R., Möller, T., & Tabrizi, S. J.
- Venue
- The Journal of Experimental Medicine
- Publisher
- Rockefeller University Press
- Published
- 2008-07-14
- Volume
- 205
- Issue
- 8
- Pages
- 1869-1877
- ISSN
- 0022-1007 (ISSN-L)
- 1540-9538
- OpenAlex
- W2115999215
- Cited by
- 650
- References
- 5
- FWCI
- 9.97
- Type
- journal article
- Language
- en
- Open access
- green
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- Björkqvist, M., Wild, E. J., Thiele, J., Silvestroni, A., Andre, R., Lahiri, N., Raibon, E., Lee, R. V., Benn, C. L., Soulet, D., Magnusson, A., Woodman, B., Landles, C., Pouladi, M. A., Hayden, M. R., Khalili-Shirazi, A., Lowdell, M. W., Brundin, P., Bates, G. P., . . . Tabrizi, S. J. (2008). A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. The Journal of Experimental Medicine. https://doi.org/10.1084/jem.20080178
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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