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CAG repeat number governs the development rate of pathology in Huntington's disease
Abstract
We compared the number of CAG repeats, the age at death, and the severity of neuropathology in 89 Huntington's disease brains. We found a linear correlation between the CAG repeat number and the quotient of the degree of atrophy in the striatum (the brain region most severely affected in Huntington's disease) divided by age at death, with an intercept at 35.5 repeats. The largest CAG repeat length, therefore, at which no pathology is expected to develop is 35.5. These results imply that striatal damage in Huntington's disease is almost entirely a lineaar function of the length of the polyglutamine stretch beyond 35.5 glutamines multiplied by the age of the patient. Thus, it is predicted that the pathological process develops linearly from birth. Analysis of other measures of striatal function could test this hypothesis and might determine when treatment for CAG repeat diseases should start.
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Cited by
Bates, 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-citationHuntington 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
Tabrizi, S. J., Langbehn, D. R., Leavitt, B. R., Roos, R. A., Durr, A., Craufurd, D., Kennard, C., Hicks, S. L., Fox, N. C., Scahill, R. I., et al. (2009) · The Lancet Neurology · doi:10.1016/s1474-4422(09)70170-x · Cited by 984
Targeting Huntingtin Expression in Patients with Huntington’s Disease
Tabrizi, S. J., Leavitt, B. R., Landwehrmeyer, G. B., Wild, E. J., Saft, C., Barker, R. A., Blair, N. F., Craufurd, D., Priller, J., Rickards, H., et al. (2019) · New England Journal of Medicine · doi:10.1056/nejmoa1900907 · Cited by 708
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
Metadata
- Authors
- Penney, J. B., Vonsattel, J., Macdonald, M. E., Gusella, J. F., & Myers, R. H.
- Venue
- Annals of Neurology
- Publisher
- Wiley
- Published
- 1997-05
- Volume
- 41
- Issue
- 5
- Pages
- 689-692
- ISSN
- 0364-5134 (ISSN-L)
- 1531-8249
- OpenAlex
- W2078320135
- Cited by
- 669
- References
- 0
- FWCI
- 3.80
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: CAG repeat number governs the development rate of pathology in Huntington's disease. https://app.cochat.ai/papers/10.1002/ana.410410521
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- Penney, J. B., Vonsattel, J., Macdonald, M. E., Gusella, J. F., & Myers, R. H. (1997). CAG repeat number governs the development rate of pathology in Huntington's disease. Annals of Neurology. https://doi.org/10.1002/ana.410410521
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