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Accumulation of dipeptide repeat proteins predates that of TDP ‐43 in frontotemporal lobar degeneration associated with hexanucleotide repeat expansions in C9ORF72 gene
Atik Baborie, Timothy D. Griffiths, Evelyn Jaros, Robert Perry, Ian G. McKeith, David J. Burn, Masami Masuda‐Suzukake, Masato Hasegawa et al.. Neuropathology and Applied Neurobiology, 2015-04-30
Topic: Disease mechanisms in lab and animal studies
Three Newcastle patients bearing an expansion in C9ORF72 were identified. These three patients died prematurely, two from bronchopneumonia within 10 months and 3 years of onset, and one from myocardial infarction 3 years after onset. In all three, DPR were plentiful throughout all cerebral cortical regions, hippocampus and cerebellum, but TDP ‐43 pathological changes were sparse. The severity of DPR pathological changes in these three patients was similar to that in the M anchester series, although the extent of TDP ‐43 pathology was significantly less. Widespread accumulation of DPR within nerve cells may occur much earlier than that of TDP ‐43 in patients with FTLD bearing expansion in C9ORF72 .
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APA 7th
Baborie, A., Griffiths, T. D., Jaros, E., Perry, R., McKeith, I. G., Burn, D. J., et al. (2015). Accumulation of dipeptide repeat proteins predates that of TDP ‐43 in frontotemporal lobar degeneration associated with hexanucleotide repeat expansions in C9ORF72 gene. Neuropathology and Applied Neurobiology. https://doi.org/10.1111/nan.12178
Vancouver
Baborie A, Griffiths TD, Jaros E, Perry R, McKeith IG, Burn DJ, et al. Accumulation of dipeptide repeat proteins predates that of TDP ‐43 in frontotemporal lobar degeneration associated with hexanucleotide repeat expansions in C9ORF72 gene. Neuropathology and Applied Neurobiology. 2015. doi:10.1111/nan.12178.
BibTeX
@article{baborie2015-60de3efb,
author = {Baborie, Atik and Griffiths, Timothy D. and Jaros, Evelyn and Perry, Robert and McKeith, Ian G. and Burn, David J. and Masuda‐Suzukake, Masami and Hasegawa, Masato and others},
title = {{Accumulation of dipeptide repeat proteins predates that of TDP ‐43 in frontotemporal lobar degeneration associated with hexanucleotide repeat expansions in C9ORF72 gene}},
journal = {Neuropathology and Applied Neurobiology},
year = {2015},
doi = {10.1111/nan.12178},
url = {https://doi.org/10.1111/nan.12178}
}
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Key findings
- Three Newcastle patients bearing an expansion in C9ORF72 were identified.
- These three patients died prematurely, two from bronchopneumonia within 10 months and 3 years of onset, and one from myocardial infarction 3 years after onset.
- In all three, DPR were plentiful throughout all cerebral cortical regions, hippocampus and cerebellum, but TDP ‐43 pathological changes were sparse.
- The severity of DPR pathological changes in these three patients was similar to that in the M anchester series, although the extent of TDP ‐43 pathology was significantly less.
- Widespread accumulation of DPR within nerve cells may occur much earlier than that of TDP ‐43 in patients with FTLD bearing expansion in C9ORF72 .
Study details
- Evidence strengthLow
How this study scored 36/100
Published in a peer-reviewed journal. Scored zero for study design, sample size, effect reported, studied in people and randomised / blinded because the record does not state it. We have no data on journal's citation record, times cited, citations per year, impact for its field and year, cited by studies here, still being cited, senior author's track record, institution's track record, multi-centre collaboration and free to read, so those were left out: it earned 20 of the 56 points we could judge it on, which is 36 out of 100.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Neuropathology and Applied Neurobiology) |
| Journal's citation record | — / 4 | No journal record from OpenAlex — left out of this score |
| 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 | 2015 — within 20 year(s) of the newest study here |
| 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 | Not stated whether this was studied in people — scores zero (not stated in the record — scored 0) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | — / 7 | No citation count (not found in OpenAlex) — left out of this score |
| Citations per year | — / 5 | No citation count or no year — left out of this score |
| Impact for its field and year | — / 6 | No field-weighted citation impact (OpenAlex has none yet) — left out of this score |
| Cited by studies here | — / 6 | Not yet checked against this library's citation graph — left out of this score |
| Still being cited | — / 4 | No yearly citation counts — left out of this score |
| Senior author's track record | — / 4 | Senior author's record not available — left out of this score |
| Institution's track record | — / 3 | No institution listed in OpenAlex — left out of this score |
| Multi-centre collaboration | — / 3 | Institutions not listed — left out of this score |
| Indexed in PubMed | 1 / 2 | Not in PubMed, but has a DOI |
| Free to read | — / 2 | Access not recorded — left out of this score |
| Points | 20 / 56 | Scored on 10 of 20 parts. We have no data for the rest, so they are left out: 20 of the 56 points we could judge it on is 36 out of 100. |
| Total | 36 / 100 | — |
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