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Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease
Turmaine M, Raza A, Mahal A, Mangiarini L, Bates GP, Davies SW.. Proceedings of the National Academy of Sciences of the United States of America, 2000-07-01
Topic: Brain proteins, microglia and lab models
Huntington's disease (HD) is a fatal inherited neurodegenerative disorder characterized by personality changes, motor impairment, and subcortical dementia. HD is one of a number of diseases caused by expression of an expanded polyglutamine repeat. We have developed several lines of mice that are transgenic for exon 1 of the HD gene containing an expanded CAG sequence.
Cite this study APA, Vancouver, BibTeX · download .bib or .ris
APA 7th
Turmaine, M., Raza, A., Mahal, A., Mangiarini, L., Bates, G. P., & Davies, S. W. (2000). Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease. Proceedings of the National Academy of Sciences of the United States of America. https://doi.org/10.1073/pnas.110078997
Vancouver
Turmaine M, Raza A, Mahal A, Mangiarini L, Bates GP, Davies SW. Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease. Proceedings of the National Academy of Sciences of the United States of America. 2000. doi:10.1073/pnas.110078997. PMID: 10869421.
BibTeX
@article{turmaine2000-10869421,
author = {Turmaine, M. and Raza, A. and Mahal, A. and Mangiarini, L. and Bates, G. P. and Davies, S. W.},
title = {{Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease}},
journal = {Proceedings of the National Academy of Sciences of the United States of America},
year = {2000},
doi = {10.1073/pnas.110078997},
pmid = {10869421},
url = {https://doi.org/10.1073/pnas.110078997}
}
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Key findings
- HD is one of a number of diseases caused by expression of an expanded polyglutamine repeat.
- We have developed several lines of mice that are transgenic for exon 1 of the HD gene containing an expanded CAG sequence.
- These mice exhibit a defined neurological phenotype along with neuronal changes that are pathognomonic for the disease.
- We have previously observed the appearance of neuronal intranuclear inclusions, but did not find evidence for neurodegeneration.
Why it matters
This study describes transgenic mouse lines for Huntington's disease that show a neurological phenotype and neuronal changes typical of the disease, without evidence of neurodegeneration. It is an animal model of Huntington's disease and does not address treating, curing, or preventing Alzheimer's disease.
Study details
- Evidence strengthLow
How this study scored 45/100
Published in a peer-reviewed journal. Scored zero for study design, sample size, effect reported and randomized / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Proceedings of the National Academy of Sciences of the United States of America) |
| Journal's citation record | 4 / 4 | Proceedings of the National Academy of Sciences: journal h-index 1,167 (OpenAlex, 2026-10-29) |
| Study design | 0 / 12 | No study design recorded — scores zero, not guessed (not stated in the record — scored 0) |
| Strength of evidence | 1 / 5 | Rated “low” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 1 / 5 | 2000 — older than 20 years, still counted |
| Sample size | 0 / 4 | Participant count not stated — scores zero (not stated in the record — scored 0) |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 0 / 3 | Laboratory or animal study (title names “transgenic”) — scores zero |
| Randomized / blinded | 0 / 2 | No randomization or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 6 / 7 | Cited 430 times (OpenAlex, 2026-10-10) |
| Citations per year | 3 / 5 | 15.4 citations a year over 28 year(s) since 2000 |
| Impact for its field and year | 4 / 6 | Cited 12.9× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 1 / 6 | Cited by 1 other studies in this library |
| Still being cited | 1 / 4 | Cited 14 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 2 / 4 | Last-listed author Stephen W. Davies: h-index 29 (OpenAlex, whole career) |
| Institution's track record | 2 / 3 | University College London: institution h-index 2,029 (OpenAlex) |
| Multicenter collaboration | 1 / 3 | OpenAlex lists 2 institution(s) in 1 country |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 10869421) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 45 / 100 | — |
What each of these 20 components measures, and what this score is not →
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